Polypeptide chains, consisting of 211 to 217 amino acid residues and having a molecular weight of approximately 22 kDa. There are two major types of light chains, kappa and lambda. Two Ig light chains and two Ig heavy chains (IMMUNOGLOBULIN HEAVY CHAINS) make one immunoglobulin molecule.
One of the types of light chains of the immunoglobulins with a molecular weight of approximately 22 kDa.
One of the types of light chain subunits of the immunoglobulins with a molecular weight of approximately 22 kDa.
Genes and gene segments encoding the IMMUNOGLOBULIN LIGHT CHAINS. Gene segments of the light chain genes are designated as V (variable), J (joining), and C (constant).
A group of sporadic, familial and/or inherited, degenerative, and infectious disease processes, linked by the common theme of abnormal protein folding and deposition of AMYLOID. As the amyloid deposits enlarge they displace normal tissue structures, causing disruption of function. Various signs and symptoms depend on the location and size of the deposits.
Ordered rearrangement of B-lymphocyte variable gene regions coding for the kappa or lambda IMMUNOGLOBULIN LIGHT CHAINS, thereby contributing to antibody diversity. It occurs during the second stage of differentiation of the IMMATURE B-LYMPHOCYTES.
An abnormal protein with unusual thermosolubility characteristics that is found in the urine of patients with MULTIPLE MYELOMA.
Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses.
Genes encoding the different subunits of the IMMUNOGLOBULINS, for example the IMMUNOGLOBULIN LIGHT CHAIN GENES and the IMMUNOGLOBULIN HEAVY CHAIN GENES. The heavy and light immunoglobulin genes are present as gene segments in the germline cells. The completed genes are created when the segments are shuffled and assembled (B-LYMPHOCYTE GENE REARRANGEMENT) during B-LYMPHOCYTE maturation. The gene segments of the human light and heavy chain germline genes are symbolized V (variable), J (joining) and C (constant). The heavy chain germline genes have an additional segment D (diversity).
That region of the immunoglobulin molecule that varies in its amino acid sequence and composition, and comprises the binding site for a specific antigen. It is located at the N-terminus of the Fab fragment of the immunoglobulin. It includes hypervariable regions (COMPLEMENTARITY DETERMINING REGIONS) and framework regions.
The domains of the immunoglobulin molecules that are invariable in their amino acid sequence within any class or subclass of immunoglobulin. They confer biological as well as structural functions to immunoglobulins. One each on both the light chains and the heavy chains comprises the C-terminus half of the IMMUNOGLOBULIN FAB FRAGMENT and two or three of them make up the rest of the heavy chains (all of the IMMUNOGLOBULIN FC FRAGMENT)
An immunolglobulin light chain-like protein composed of an IMMUNOGLOBULIN VARIABLE REGION-like peptide (such as light chain like lambda5 peptide) and an IMMUNOGLOBULIN CONSTANT REGION-like peptide (such as Vpreb1 peptide). Surrogate light chains associate with MU IMMUNOGLOBULIN HEAVY CHAINS in place of a conventional immunoglobulin light chains to form pre-B cell receptors.
Abnormal immunoglobulins characteristic of MULTIPLE MYELOMA.
The largest of polypeptide chains comprising immunoglobulins. They contain 450 to 600 amino acid residues per chain, and have molecular weights of 51-72 kDa.
Any discrete, presumably solitary, mass of neoplastic PLASMA CELLS either in BONE MARROW or various extramedullary sites.
A fibrous protein complex that consists of proteins folded into a specific cross beta-pleated sheet structure. This fibrillar structure has been found as an alternative folding pattern for a variety of functional proteins. Deposits of amyloid in the form of AMYLOID PLAQUES are associated with a variety of degenerative diseases. The amyloid structure has also been found in a number of functional proteins that are unrelated to disease.
The smaller subunits of MYOSINS that bind near the head groups of MYOSIN HEAVY CHAINS. The myosin light chains have a molecular weight of about 20 KDa and there are usually one essential and one regulatory pair of light chains associated with each heavy chain. Many myosin light chains that bind calcium are considered "calmodulin-like" proteins.
A group of elongate elasmobranchs. Sharks are mostly marine fish, with certain species large and voracious.
The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.
A class of immunoglobulin bearing mu chains (IMMUNOGLOBULIN MU-CHAINS). IgM can fix COMPLEMENT. The name comes from its high molecular weight and originally being called a macroglobulin.
That portion of the electromagnetic spectrum in the visible, ultraviolet, and infrared range.
A malignancy of mature PLASMA CELLS engaging in monoclonal immunoglobulin production. It is characterized by hyperglobulinemia, excess Bence-Jones proteins (free monoclonal IMMUNOGLOBULIN LIGHT CHAINS) in the urine, skeletal destruction, bone pain, and fractures. Other features include ANEMIA; HYPERCALCEMIA; and RENAL INSUFFICIENCY.
The phenomenon of immense variability characteristic of ANTIBODIES. It enables the IMMUNE SYSTEM to react specifically against the essentially unlimited kinds of ANTIGENS it encounters. Antibody diversity is accounted for by three main theories: (1) the Germ Line Theory, which holds that each antibody-producing cell has genes coding for all possible antibody specificities, but expresses only the one stimulated by antigen; (2) the Somatic Mutation Theory, which holds that antibody-producing cells contain only a few genes, which produce antibody diversity by mutation; and (3) the Gene Rearrangement Theory, which holds that antibody diversity is generated by the rearrangement of IMMUNOGLOBULIN VARIABLE REGION gene segments during the differentiation of the ANTIBODY-PRODUCING CELLS.
A group of related diseases characterized by an unbalanced or disproportionate proliferation of immunoglobulin-producing cells, usually from a single clone. These cells frequently secrete a structurally homogeneous immunoglobulin (M-component) and/or an abnormal immunoglobulin.
Lymphoid cells concerned with humoral immunity. They are short-lived cells resembling bursa-derived lymphocytes of birds in their production of immunoglobulin upon appropriate stimulation.
Represents 15-20% of the human serum immunoglobulins, mostly as the 4-chain polymer in humans or dimer in other mammals. Secretory IgA (IMMUNOGLOBULIN A, SECRETORY) is the main immunoglobulin in secretions.
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
A segment of the immunoglobulin heavy chains, encoded by the IMMUNOGLOBULIN HEAVY CHAIN GENES in the J segment where, during the maturation of B-LYMPHOCYTES; the gene segment for the variable region upstream is joined to a constant region gene segment downstream. The exact position of joining of the two gene segments is variable and contributes to ANTIBODY DIVERSITY. It is distinguished from the IMMUNOGLOBULIN J CHAINS; a separate polypeptide that serves as a linkage piece in polymeric IGA or IGM.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
A group of disorders having a benign course but exhibiting clinical and histological features suggestive of malignant lymphoma. Pseudolymphoma is characterized by a benign infiltration of lymphoid cells or histiocytes which microscopically resembles a malignant lymphoma. (From Dorland, 28th ed & Stedman, 26th ed)
A 15 kD "joining" peptide that forms one of the linkages between monomers of IMMUNOGLOBULIN A or IMMUNOGLOBULIN M in the formation of polymeric immunoglobulins. There is one J chain per one IgA dimer or one IgM pentamer. It is also involved in binding the polymeric immunoglobulins to POLYMERIC IMMUNOGLOBULIN RECEPTOR which is necessary for their transcytosis to the lumen. It is distinguished from the IMMUNOGLOBULIN JOINING REGION which is part of the IMMUNOGLOBULIN VARIABLE REGION of the immunoglobulin light and heavy chains.
The class of heavy chains found in IMMUNOGLOBULIN M. They have a molecular weight of approximately 72 kDa and they contain about 57 amino acid residues arranged in five domains and have more oligosaccharide branches and a higher carbohydrate content than the heavy chains of IMMUNOGLOBULIN G.
The ordered rearrangement of gene regions by DNA recombination such as that which occurs normally during development.
Ordered rearrangement of B-lymphocyte variable gene regions coding for the IMMUNOGLOBULIN CHAINS, thereby contributing to antibody diversity. It occurs during the differentiation of the IMMATURE B-LYMPHOCYTES.
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
IMMUNOGLOBULINS on the surface of B-LYMPHOCYTES. Their MESSENGER RNA contains an EXON with a membrane spanning sequence, producing immunoglobulins in the form of type I transmembrane proteins as opposed to secreted immunoglobulins (ANTIBODIES) which do not contain the membrane spanning segment.
Partial immunoglobulin molecules resulting from selective cleavage by proteolytic enzymes or generated through PROTEIN ENGINEERING techniques.
A lymphoproliferative disorder characterized by pleomorphic B-LYMPHOCYTES including PLASMA CELLS, with increased levels of monoclonal serum IMMUNOGLOBULIN M. There is lymphoplasmacytic cells infiltration into bone marrow and often other tissues, also known as lymphoplasmacytic lymphoma. Clinical features include ANEMIA; HEMORRHAGES; and hyperviscosity.
A hereditary or acquired form of generalized dysfunction of the PROXIMAL KIDNEY TUBULE without primary involvement of the KIDNEY GLOMERULUS. It is usually characterized by the tubular wasting of nutrients and salts (GLUCOSE; AMINO ACIDS; PHOSPHATES; and BICARBONATES) resulting in HYPOKALEMIA; ACIDOSIS; HYPERCALCIURIA; and PROTEINURIA.
Specialized forms of antibody-producing B-LYMPHOCYTES. They synthesize and secrete immunoglobulin. They are found only in lymphoid organs and at sites of immune responses and normally do not circulate in the blood or lymph. (Rosen et al., Dictionary of Immunology, 1989, p169 & Abbas et al., Cellular and Molecular Immunology, 2d ed, p20)
An enzyme that phosphorylates myosin light chains in the presence of ATP to yield myosin-light chain phosphate and ADP, and requires calcium and CALMODULIN. The 20-kDa light chain is phosphorylated more rapidly than any other acceptor, but light chains from other myosins and myosin itself can act as acceptors. The enzyme plays a central role in the regulation of smooth muscle contraction.
An excess of GAMMA-GLOBULINS in the serum due to chronic infections or PARAPROTEINEMIAS.
Electrophoresis applied to BLOOD PROTEINS.
A group of heterogeneous lymphoid tumors generally expressing one or more B-cell antigens or representing malignant transformations of B-lymphocytes.
A general term for various neoplastic diseases of the lymphoid tissue.
In vitro method for producing large amounts of specific DNA or RNA fragments of defined length and sequence from small amounts of short oligonucleotide flanking sequences (primers). The essential steps include thermal denaturation of the double-stranded target molecules, annealing of the primers to their complementary sequences, and extension of the annealed primers by enzymatic synthesis with DNA polymerase. The reaction is efficient, specific, and extremely sensitive. Uses for the reaction include disease diagnosis, detection of difficult-to-isolate pathogens, mutation analysis, genetic testing, DNA sequencing, and analyzing evolutionary relationships.
Local surface sites on antibodies which react with antigen determinant sites on antigens (EPITOPES.) They are formed from parts of the variable regions of FAB FRAGMENTS.
Three regions (CDR1; CDR2 and CDR3) of amino acid sequence in the IMMUNOGLOBULIN VARIABLE REGION that are highly divergent. Together the CDRs from the light and heavy immunoglobulin chains form a surface that is complementary to the antigen. These regions are also present in other members of the immunoglobulin superfamily, for example, T-cell receptors (RECEPTORS, ANTIGEN, T-CELL).
Immunoglobulin preparations used in intravenous infusion, containing primarily IMMUNOGLOBULIN G. They are used to treat a variety of diseases associated with decreased or abnormal immunoglobulin levels including pediatric AIDS; primary HYPERGAMMAGLOBULINEMIA; SCID; CYTOMEGALOVIRUS infections in transplant recipients, LYMPHOCYTIC LEUKEMIA, CHRONIC; Kawasaki syndrome, infection in neonates, and IDIOPATHIC THROMBOCYTOPENIC PURPURA.
Chemical analysis based on the phenomenon whereby light, passing through a medium with dispersed particles of a different refractive index from that of the medium, is attenuated in intensity by scattering. In turbidimetry, the intensity of light transmitted through the medium, the unscattered light, is measured. In nephelometry, the intensity of the scattered light is measured, usually, but not necessarily, at right angles to the incident light beam.
A tetrameric protein, molecular weight between 50,000 and 70,000, consisting of 4 equal chains, and migrating on electrophoresis in 3 fractions more mobile than serum albumin. Its concentration ranges from 7 to 33 per cent in the serum, but levels decrease in liver disease.
A diverse superfamily of proteins that function as translocating proteins. They share the common characteristics of being able to bind ACTINS and hydrolyze MgATP. Myosins generally consist of heavy chains which are involved in locomotion, and light chains which are involved in regulation. Within the structure of myosin heavy chain are three domains: the head, the neck and the tail. The head region of the heavy chain contains the actin binding domain and MgATPase domain which provides energy for locomotion. The neck region is involved in binding the light-chains. The tail region provides the anchoring point that maintains the position of the heavy chain. The superfamily of myosins is organized into structural classes based upon the type and arrangement of the subunits they contain.
A group of genetically identical cells all descended from a single common ancestral cell by mitosis in eukaryotes or by binary fission in prokaryotes. Clone cells also include populations of recombinant DNA molecules all carrying the same inserted sequence. (From King & Stansfield, Dictionary of Genetics, 4th ed)
Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.
A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms.
The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. PROTEIN STRUCTURE, QUATERNARY describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain).
Established cell cultures that have the potential to propagate indefinitely.
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
Common name for the species Gallus gallus, the domestic fowl, in the family Phasianidae, order GALLIFORMES. It is descended from the red jungle fowl of SOUTHEAST ASIA.
The species Oryctolagus cuniculus, in the family Leporidae, order LAGOMORPHA. Rabbits are born in burrows, furless, and with eyes and ears closed. In contrast with HARES, rabbits have 22 chromosome pairs.
A genus of gram-positive, anaerobic, coccoid bacteria that is part of the normal flora of humans. Its organisms are opportunistic pathogens causing bacteremias and soft tissue infections.
The rate dynamics in chemical or physical systems.
A deoxyribonucleotide polymer that is the primary genetic material of all cells. Eukaryotic and prokaryotic organisms normally contain DNA in a double-stranded state, yet several important biological processes transiently involve single-stranded regions. DNA, which consists of a polysugar-phosphate backbone possessing projections of purines (adenine and guanine) and pyrimidines (thymine and cytosine), forms a double helix that is held together by hydrogen bonds between these purines and pyrimidines (adenine to thymine and guanine to cytosine).
Antibodies produced by a single clone of cells.
Electrophoresis in which a pH gradient is established in a gel medium and proteins migrate until they reach the site (or focus) at which the pH is equal to their isoelectric point.
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
A change from planar to elliptic polarization when an initially plane-polarized light wave traverses an optically active medium. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
A chronic leukemia characterized by abnormal B-lymphocytes and often generalized lymphadenopathy. In patients presenting predominately with blood and bone marrow involvement it is called chronic lymphocytic leukemia (CLL); in those predominately with enlarged lymph nodes it is called small lymphocytic lymphoma. These terms represent spectrums of the same disease.
Widely used technique which exploits the ability of complementary sequences in single-stranded DNAs or RNAs to pair with each other to form a double helix. Hybridization can take place between two complimentary DNA sequences, between a single-stranded DNA and a complementary RNA, or between two RNA sequences. The technique is used to detect and isolate specific sequences, measure homology, or define other characteristics of one or both strands. (Kendrew, Encyclopedia of Molecular Biology, 1994, p503)
An immunoglobulin associated with MAST CELLS. Overexpression has been associated with allergic hypersensitivity (HYPERSENSITIVITY, IMMEDIATE).
The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.
The sum of the weight of all the atoms in a molecule.
The principle immunoglobulin in exocrine secretions such as milk, respiratory and intestinal mucin, saliva and tears. The complete molecule (around 400 kD) is composed of two four-chain units of IMMUNOGLOBULIN A, one SECRETORY COMPONENT and one J chain (IMMUNOGLOBULIN J-CHAINS).
A family of cellular proteins that mediate the correct assembly or disassembly of polypeptides and their associated ligands. Although they take part in the assembly process, molecular chaperones are not components of the final structures.
The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells.
Technique using an instrument system for making, processing, and displaying one or more measurements on individual cells obtained from a cell suspension. Cells are usually stained with one or more fluorescent dyes specific to cell components of interest, e.g., DNA, and fluorescence of each cell is measured as it rapidly transverses the excitation beam (laser or mercury arc lamp). Fluorescence provides a quantitative measure of various biochemical and biophysical properties of the cell, as well as a basis for cell sorting. Other measurable optical parameters include light absorption and light scattering, the latter being applicable to the measurement of cell size, shape, density, granularity, and stain uptake.
The arrangement of two or more amino acid or base sequences from an organism or organisms in such a way as to align areas of the sequences sharing common properties. The degree of relatedness or homology between the sequences is predicted computationally or statistically based on weights assigned to the elements aligned between the sequences. This in turn can serve as a potential indicator of the genetic relatedness between the organisms.
Diseases in which there is a familial pattern of AMYLOIDOSIS.
White blood cells formed in the body's lymphoid tissue. The nucleus is round or ovoid with coarse, irregularly clumped chromatin while the cytoplasm is typically pale blue with azurophilic (if any) granules. Most lymphocytes can be classified as either T or B (with subpopulations of each), or NATURAL KILLER CELLS.
The classes of immunoglobulins found in any species of animal. In man there are nine classes that migrate in five different groups in electrophoresis; they each consist of two light and two heavy protein chains, and each group has distinguishing structural and functional properties.
The sequential correspondence of nucleotides in one nucleic acid molecule with those of another nucleic acid molecule. Sequence homology is an indication of the genetic relatedness of different organisms and gene function.
A rigorously mathematical analysis of energy relationships (heat, work, temperature, and equilibrium). It describes systems whose states are determined by thermal parameters, such as temperature, in addition to mechanical and electromagnetic parameters. (From Hawley's Condensed Chemical Dictionary, 12th ed)
The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.
The process of cumulative change over successive generations through which organisms acquire their distinguishing morphological and physiological characteristics.
An immunoglobulin which accounts for less than 1% of plasma immunoglobulin. It is found on the membrane of many circulating B LYMPHOCYTES.
Histochemical localization of immunoreactive substances using labeled antibodies as reagents.
Heavy chains of IMMUNOGLOBULIN G having a molecular weight of approximately 51 kDa. They contain about 450 amino acid residues arranged in four domains and an oligosaccharide component covalently bound to the Fc fragment constant region. The gamma heavy chain subclasses (for example, gamma 1, gamma 2a, and gamma 2b) of the IMMUNOGLOBULIN G isotype subclasses (IgG1, IgG2A, and IgG2B) resemble each other more closely than the heavy chains of the other IMMUNOGLOBULIN ISOTYPES.
Univalent antigen-binding fragments composed of one entire IMMUNOGLOBULIN LIGHT CHAIN and the amino terminal end of one of the IMMUNOGLOBULIN HEAVY CHAINS from the hinge region, linked to each other by disulfide bonds. Fab contains the IMMUNOGLOBULIN VARIABLE REGIONS, which are part of the antigen-binding site, and the first IMMUNOGLOBULIN CONSTANT REGIONS. This fragment can be obtained by digestion of immunoglobulins with the proteolytic enzyme PAPAIN.
Crystallizable fragments composed of the carboxy-terminal halves of both IMMUNOGLOBULIN HEAVY CHAINS linked to each other by disulfide bonds. Fc fragments contain the carboxy-terminal parts of the heavy chain constant regions that are responsible for the effector functions of an immunoglobulin (COMPLEMENT fixation, binding to the cell membrane via FC RECEPTORS, and placental transport). This fragment can be obtained by digestion of immunoglobulins with the proteolytic enzyme PAPAIN.
A method (first developed by E.M. Southern) for detection of DNA that has been electrophoretically separated and immobilized by blotting on nitrocellulose or other type of paper or nylon membrane followed by hybridization with labeled NUCLEIC ACID PROBES.
Pathological processes of the KIDNEY or its component tissues.
Allelic variants of the immunoglobulin light chains (IMMUNOGLOBULIN LIGHT CHAINS) or heavy chains (IMMUNOGLOBULIN HEAVY CHAINS) encoded by ALLELES of IMMUNOGLOBULIN GENES.
Gene rearrangement of the B-lymphocyte which results in a substitution in the type of heavy-chain constant region that is expressed. This allows the effector response to change while the antigen binding specificity (variable region) remains the same. The majority of class switching occurs by a DNA recombination event but it also can take place at the level of RNA processing.
The property of antibodies which enables them to react with some ANTIGENIC DETERMINANTS and not with others. Specificity is dependent on chemical composition, physical forces, and molecular structure at the binding site.
A phosphoprotein phosphatase that is specific for MYOSIN LIGHT CHAINS. It is composed of three subunits, which include a catalytic subunit, a myosin binding subunit, and a third subunit of unknown function.
The meaning ascribed to the BASE SEQUENCE with respect to how it is translated into AMINO ACID SEQUENCE. The start, stop, and order of amino acids of a protein is specified by consecutive triplets of nucleotides called codons (CODON).

Induction of Ig light chain gene rearrangement in heavy chain-deficient B cells by activated Ras. (1/1652)

During B cell development, rearrangement and expression of Ig heavy chain (HC) genes promote development and expansion of pre-B cells accompanied by the onset of Ig light chain (LC) variable region gene assembly. To elucidate the signaling pathways that control these events, we have tested the ability of activated Ras expression to promote B cell differentiation to the stage of LC gene rearrangement in the absence of Ig HC gene expression. For this purpose, we introduced an activated Ras expression construct into JH-deleted embryonic stem cells that lack the ability to assemble HC variable region genes and assayed differentiation potential by recombination activating gene (RAG) 2-deficient blastocyst complementation. We found that activated Ras expression induces the progression of B lineage cells beyond the developmental checkpoint ordinarily controlled by mu HC. Such Ras/JH-deleted B cells accumulate in the periphery but continue to express markers associated with precursor B cells including RAG gene products. These peripheral Ras/JH-deleted B cell populations show extensive Ig LC gene rearrangement but maintain an extent of kappa LC gene rearrangement and a preference for kappa over lambda LC gene rearrangement similar to that of wild-type B cells. We discuss these findings in the context of potential mechanisms that may regulate Ig LC gene rearrangement.  (+info)

Characterization of an immunoglobin cDNA clone containing the variable and constant regions for the MOPC 21 kappa light chain. (2/1652)

Nucleotide sequence analysis and restriction endonuclease mapping have been used to characterize a cDNA copy of immunoglobulin MOPC 21 Kappa mRNA clones in the bacterial plasmid pMB9. Three regions of the inserted cDNA of plasmid pL21-1 have been sequenced and match the known protein sequence at amino acid residues 1-24, 128-138 and 171-179. With these sequences to provide absolute correlations between the restriction map and the structural gene sequence it has been possible to exactly deduce the positions of all 11 of the insert restriction sites mapped within the structural gene. The pL21-1 insert contains the complete variable and constant regions as well as parts of the 3' untranslated and polypeptide leader coding sequences.  (+info)

Recombinant DNA clones constructed from immunoglobulin kappa light chain messenger RNA. (3/1652)

Recombinant DNA clones have been generated from mouse myeloma MOPC 21 immunoglobulin kappa light chain mRNA. Complementary DNA (cDNA) synthesized on kappa light chain mRNA by reverse transcriptase was made double stranded and inserted into the bacterial plasmid vector, pMB9. Approximately 70 tetracycline-resistant transformed colonies containing kappa light chain mRNA sequences were identified by colony hybridization. Five of these recombinant clones were selected and characterized. Three clones contain both kappa light chain constant and variable region sequences. Two of these three recombinant clones have been shown to include all of the kappa light chain constant and variable region coding sequences. Another of the five selected recombinant clones contain kappa light chain constant region sequences. The remaining characterized clone appears to be derived from sequences at the 5'-end of kappa light chain mRNA, possibly extending to the terminal cap structure.  (+info)

Novel mechanisms control the folding and assembly of lambda5/14.1 and VpreB to produce an intact surrogate light chain. (4/1652)

Surrogate light chain, which escorts the mu heavy chain to the cell surface, is a critical component of the pre-B cell receptor complex. The two proteins that comprise the surrogate light chain, VpreB and lambda5/14.1, contain both unique regions and Ig-like domains. The unique regions have been postulated to function in the assembly of the surrogate light chain. However, by using transient transfection of COS7 cells, we show that deletion of the unique regions of both proteins did not inhibit the assembly of surrogate light chain. Instead, in vivo folding studies showed that the unique region of lambda5/14.1 acts as an intramolecular chaperone by preventing the folding of this protein when it is expressed in the absence of its partner, VpreB. The Ig domains of both lambda5/14.1 and VpreB are atypical. The one in VpreB lacks one of the canonical beta strands whereas the one in lambda5/14.1 has an extra beta strand. Deletion of the extra beta strand in lambda5/14.1 completely abrogated the formation of the surrogate light chain, demonstrating that complementation of the incomplete Ig domain in VpreB by the extra beta strand in lambda5/14.1 was necessary and sufficient for the folding and assembly of these proteins. Our studies reveal two novel mechanisms for regulating surrogate light chain formation: (i) the presence of an intramolecular chaperone that prevents folding of the unassembled subunit but that remains part of the mature assembled protein, and (ii) splitting an Ig domain between two proteins to control their folding and assembly.  (+info)

Assignment of genes for immunoglobulin kappa and heavy chains to chromosomes 6 and 12 in mouse. (5/1652)

Using somatic cell hybrids from fusions of lymphocytes of two different mouse stocks with the myeloma cell line X63-Ag8, we have assigned genes for the immunoglobulin heavy and kappa-type light chains to chromosomes 12 and 6, respectively. The two mouse stocks exhibit karyotypes consisting of nine pairs of metacentric chromosomes as a result of centric fusions of acrocentric chromosomes in different combinations. In the hybrid cells these metacentric chromosomes can be distinguished from the acrocentric chromosomes of myeloma origin, permitting correlation of Ig chain expression with mitotic loss of individual metacentric chromosomes.  (+info)

The structure of an entire noncovalent immunoglobulin kappa light-chain dimer (Bence-Jones protein) reveals a weak and unusual constant domains association. (6/1652)

Monoclonal free light chains secreted in immunoproliferative disorders are frequently involved in renal complications, including a specific proximal tubule impairment, Fanconi's syndrome. The latter is characterized in most cases by intracellular crystallization including a light-chain variable-domain fragment which resists lysosomal proteases. Bence-Jones protein (BJP) DEL was isolated from a patient with myeloma-associated Fanconi's syndrome. The crystal structure of this human kappa immunoglobulin light-chain noncovalent dimer was determined using molecular replacement with the structure of molecule REI, as the variable domain, and that of BJP LOC as the constant domain. To our knowledge, DEL is the first complete kappa BJP structure described to date. The R-factor is 20.7% at 2.8 A resolution. The BJP DEL dimer was compared with other light-chain dimers and with Fab fragments with a kappa light chain. Although the domain-folding pattern was similar, the relative positions of the constant domains differed. BJP DEL showed a noncanonical quaternary structural arrangement which may be attributable to the poor CL-CL affinity and lack of an interchain disulfide bridge, combined with the conformational editing effect of the crystal-packing forces. Our results suggest that, in the absence of a disulfide bridge, most BJP CLs are probably mobile in solution. This may explain their high susceptibility to proteases and the absence of naturally occurring crystals for these dimers. Furthermore, these findings of an unusual quaternary structure of an immunoglobulin light-chain association extend our knowledge about the large and highly diverse structures of the immunoglobulin superfamily.  (+info)

Physicochemical consequences of amino acid variations that contribute to fibril formation by immunoglobulin light chains. (7/1652)

The most common form of systemic amyloidosis originates from antibody light chains. The large number of amino acid variations that distinguish amyloidogenic from nonamyloidogenic light chain proteins has impeded our understanding of the structural basis of light-chain fibril formation. Moreover, even among the subset of human light chains that are amyloidogenic, many primary structure differences are found. We compared the thermodynamic stabilities of two recombinant kappa4 light-chain variable domains (V(L)s) derived from amyloidogenic light chains with a V(L) from a benign light chain. The amyloidogenic V(L)s were significantly less stable than the benign V(L). Furthermore, only the amyloidogenic V(L)s formed fibrils under native conditions in an in vitro fibril formation assay. We used site-directed mutagenesis to examine the consequences of individual amino acid substitutions found in the amyloidogenic V(L)s on stability and fibril formation capability. Both stabilizing and destabilizing mutations were found; however, only destabilizing mutations induced fibril formation in vitro. We found that fibril formation by the benign V(L) could be induced by low concentrations of a denaturant. This indicates that there are no structural or sequence-specific features of the benign V(L) that are incompatible with fibril formation, other than its greater stability. These studies demonstrate that the V(L) beta-domain structure is vulnerable to destabilizing mutations at a number of sites, including complementarity determining regions (CDRs), and that loss of variable domain stability is a major driving force in fibril formation.  (+info)

Cells with clonal light chains are present in peripheral blood at diagnosis and in apheretic stem cell harvests of primary amyloidosis. (8/1652)

In primary systemic amyloidosis, small numbers of bone marrow plasma cells secrete monoclonal light chains that form extracellular fibrils (amyloid) in various organs. Evidence limited to a few cases suggests that rare clonal elements can also be found in the peripheral blood (PB), and this may be relevant in PB stem cell autotransplantation. Since up to 40% of amyloid clones do not synthesize heavy chains, in order to detect tumor cells with high specificity and sensitivity we developed a seminested allele-specific oligonucleotide polymerase chain reaction for tumor light chains. Clone-related sequences were detected in DNA and/or cDNA from the PB cells of eight of 10 patients at diagnosis and from apheretic collections of three of four cases undergoing PB progenitor autotransplantation. Since there are experimental data suggesting that circulating tumor cells may be involved in the growth of the amyloidogenic clone and may be chemoresistant, these findings are relevant to the use of leukapheresis purging strategies for PB progenitor autotransplantation in amyloidosis.  (+info)

Background: Immunoglobulin free light chains (FLC) form the substrate for synthesis of amyloid fibrils in patients with AL amyloidosis. Development of FLC assay (Freelite) has allowed us to better assess the clonal cell burden in patients with AL amyloid. The relationship of the light chain types and their levels in serum to the clinical feature at presentation and the eventual outcome has not been systematically studied.. Methods: We identified 730 patients with biopsy proven AL amyloidosis, who were seen at Mayo Clinic between January 1980 and July 2006, who were seen within 90 days of their diagnosis and in whom FLC levels were performed or had stored serum available for analysis. Cardiac biomarkers (cTnT, NT ProBNP) were also performed on stored serum for some of the patients as part of previous studies. Clinical data and follow up status are prospectively collected into the Dysproteinemia database, which was used for the study.. Results: The median age of the study population was 63 years ...
TY - JOUR. T1 - Tyrosine residues mediate fibril formation in a dynamic light chain dimer interface. AU - DiCostanzo, Ara Celi. AU - Thompson, James R.. AU - Peterson, Francis C.. AU - Volkman, Brian F.. AU - Ramirez-Alvarado, Marina. PY - 2012/8/10. Y1 - 2012/8/10. N2 - Light chain amyloidosis is an incurable protein misfolding disease where monoclonal immunoglobulin light chains misfold and deposit as amyloid fibrils, causing organ failure and death. Previously, we determined that amyloidogenic light chains AL-09 and AL-103 do not form fibrils at pH 10 (tyrosine pK a). There are three tyrosine residues (32, 91, and 96) clustered in the dimer interface, interacting differently in the two light chain proteins due to their two different dimer conformations. These tyrosines may be ionized at pH 10, causing repulsion and inhibiting fibril formation. Here, we characterize single and double Tyr-to-Phe mutations in AL-09 and AL-103. All AL-09 Tyr-to-Phe mutants form fibrils at pH 10, whereas none of ...
Immunoglobulin light chain amyloidosis (AL) is characterized by a limited clonal expansion of plasma cells and amyloid formation. Here, we report restriction in the diversity of VL gene usage with a d
Serum free light chains (SFLC, kappa and lambda) are proteins produce by immune cells that are not part of whole (intact) antibodies. Lab tests measure free light chains in the blood and a kappa/lambda ratio to help diagnose multiple myeloma and related diseases.
TY - JOUR. T1 - Long-Term Outcome of Renal Transplantation in Light-Chain Deposition Disease. AU - Leung, Nelson. AU - Lager, Donna J.. AU - Gertz, Morie. AU - Wilson, Kirk. AU - Kanakiriya, Sharan. AU - Fervenza, Fernando Custodio. PY - 2004/1. Y1 - 2004/1. N2 - Background: Light-chain deposition disease (LCDD) is a monoclonal gammopathy characterized by nonamyloid deposition of light chain in various organs. A small number of kidney transplantations have been performed on LCDD patients in whom end-stage renal disease (ESRD) developed. Methods: The authors retrospectively reviewed the clinical and histologic findings and outcome of 7 patients with LCDD who underwent kidney transplantation at our institution. Results: Renal insufficiency, hypertension, and proteinuria were present in all 7 patients. Proteinuria level was greater than 3.5 g/24 h in 3 patients. Three patients had microscopic hematuria. Monoclonal protein was detected in the serum in 3 patients, urine in 5, and was undetectable in ...
Light-chain deposition disease (LCDD) is the deposition of monoclonal light chains in multiple organs. It is a rare disease characterized by deposition of nonamyloid immunoglobulin light chains, and they do not stain with Congo red and do not exhibit a fibrillar structure when examined ultrastructurally.
Immunoglobulin light chain gene translocations in non-Hodgkins lymphoma as assessed by fluorescence in situ hybridisation.: In non-Hodgkins lymphoma (NHL), th
Garver, F.A.; Chang, L.; Mendicino, J.; Isobe, T.; Osserman, E.F., 1975: Primary structure of a deleted human lambda type immunoglobulin light chain containing carbohydrate: protein Sm lambda
FRI0479 Serum Free Light Chains of Immunoglobulins Are Associated with Disease Activity in Systemic Sclerosis: A Prospective and Controlled Study ...
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This test has been cleared or approved by the U.S. Food and Drug Administration and is used per manufacturers instructions. Performance characteristics were verified by Mayo Clinic in a manner consistent with CLIA requirements ...
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Immunoglobulin secretion by plasma cells infiltrating synovial membranes is a prominent feature of RA. Previous analyses of a cDNA library generated from synovium of RA patient BC revealed immunoglobulin κ light chain transcripts with extensive somatic mutation, frequent N region addition, and unexpected variation in the lengths of CDR3 regions which form the center of the antigen binding site. To determine if these characteristics are present in other individuals, we performed reverse transcription-polymerase chain reaction amplification and sequenced ≥10 Vκ-containing amplicons from nine tissue samples: synovia of three individuals with long-standing RA (including patient BC), PBLs of two of these individuals, and PBLs or splenocytes of four normal individuals. Increased levels of somatic mutation in PBLs appeared to correlate with increased age, which may reflect accumulation of circulating memory cells and/or decreased bone marrow production of naive B lymphocytes. Two of three RA ...
Because of the less-than-robust response to therapy and impact on choice of optimal chemotherapy and prognosis, chronic kidney disease has drawn attention in the treatment of multiple myeloma, a malignant hematologic disorder that can produce significant amounts of monoclonal immunoglobulin free light chains (FLCs). These low-molecular-weight proteins are relatively freely filtered through the glomerulus and are reabsorbed by the proximal tubule. The present study demonstrated that during the process of metabolism of immunoglobulin FLCs, ROS activated the STAT1 pathway in proximal tubule epithelium. STAT1 activation served as the seminal signaling molecule that produced the proinflammatory molecule IL-1β, as well as the profibrotic agent TGF-β by this portion of the nephron. These effects occurred in vivo and were produced specifically by the generation of hydrogen peroxide by the VL domain of the light chain. To the extent that the experiments reflect the human condition, these studies offer ...
Because of the less-than-robust response to therapy and impact on choice of optimal chemotherapy and prognosis, chronic kidney disease has drawn attention in the treatment of multiple myeloma, a malignant hematologic disorder that can produce significant amounts of monoclonal immunoglobulin free light chains (FLCs). These low-molecular-weight proteins are relatively freely filtered through the glomerulus and are reabsorbed by the proximal tubule. The present study demonstrated that during the process of metabolism of immunoglobulin FLCs, ROS activated the STAT1 pathway in proximal tubule epithelium. STAT1 activation served as the seminal signaling molecule that produced the proinflammatory molecule IL-1β, as well as the profibrotic agent TGF-β by this portion of the nephron. These effects occurred in vivo and were produced specifically by the generation of hydrogen peroxide by the VL domain of the light chain. To the extent that the experiments reflect the human condition, these studies offer ...
This is an interesting study as it doesnt treat the underlying disease which is MM but is attempting to treat in an animal model a consequence of the disease. Cast nephropathy which leads to intra tubular obstruction can quickly lead to dialysis in many cases. This is a study that shows by using the CDR3 inhibitor, we prevent this bad light chain and THP combo and prevent the precipitation in the tubules. We also know that the free light chains behave differently in each MM case. In some cases, we get cast nephrpathy and in some cases we get light chain deposition disease and some amyloidosis. It appears to be characteristics of the specific light chains produced. While this method might be optimal for cast nephrpathy cases, dont know if this will prevent all MM associated renal diseases. Regardless, this study might lead to growing MM related cast nephropathy studies with this inhibitor; hopefully. ...
The comparison of UPEP and iFLC results at the time of diagnosis (baseline) showed that iFLC was a more sensitive measure of disease, with 100% of patients positive and measurable compared with 78% positive and 64% measurable by UPEP. This pattern of results was repeated with the assessments after 1 and 3 cycles of treatment and was also in accord with all other published studies of patients with confirmed diagnoses of LCMM.14,21 UPEP and iFLC were chosen as the measures for comparison as they are the methods approved for monitoring LCMM by the IMWG6 and are quantitative, in contrast to the more sensitive but nonquantitative uIFE or the more sensitive sFLC ratio which may become variable if the noninvolved light chain is highly suppressed.19 Nevertheless, achieving a normal sFLC has been shown to be a clinically relevant measure of treatment efficacy.22,23. The number of missing observations was much higher for the urine assays, particularly after cycle 1, with 60 missing, although urine ...
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Note Added in Proof to appear with the article: A Homologue of the Rabbit Kappa Chain Allotype b9 on IgG from a Cottontail Rabbit by Simonetta Landucci Tosi, Rose G. Mage, and Roberto M. Tosi appearing on page 679:. Extensive studies of the phylogeny of rabbit immunoglobulin light chain markers have recently been completed by W. van der Loo, C. Hamers-Casterman and R. Hamers, and reported in a brief communication entitled, Phylogeny of the rabbit immunoglobulin light chain markers: Distribution of determinants associated with rabbit b-locus in different lagomorph families published in Arch. Internat. Physiol. Biochim. 89, 54, 1976. A detailed report entitled, Investigations of the origin of the b-locus polymorphism of the rabbit kappa light chain is contained in the D. Sc. thesis, Vrije Universiteit Brussels by Dr. W. van der Loo, 1975. Their observations are generally in accord with ours.. ...
About 1 in 5 people with myeloma (20%) do not produce complete immunoglobulins (paraproteins). They only produce part of the immunoglobulin called the light chain (Bence Jones) protein.. The light chain proteins are smaller than complete immunoglobulins and show up in the blood and urine. This can be monitored through the traditional way of urine tests but more commonly now it is monitored in the blood through a test called serum free light chains.. This test can pick up free light chains in the blood and is easier as you dont have to collect 24 hours worth of urine every month. You may have both these tests to help diagnose and monitor this type of myeloma.. ...
Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses ...
Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses.. ...
Amyloid fibrils and partially unfolded intermediates can be distinguished serologically from native amyloidogenic precursor proteins or peptides. In this regard, we previously had reported that mAb 11-1F4, generated by immunizing mice with a thermally denatured variable domain (VL) fragment of the h …
A patient is presented in whom the amyloid component of an intracerebral amyloidoma has been purified and characterized by amino acid sequence analysis. The material originated from an autopsy of a 76-year-old man who 15 years earlier had been operated for an intracerebral amyloid tumour. The tu …
AA (Inflammatory) AmyloidosisDiabetic NephropathyFamilial Renal AmyloidosisIgA NephropathyImmunoglobulin-Related AmyloidosisLight Chain-Associated Renal DisordersMultiple MyelomaNephrotic Syndrome
Addresses: Pilstrom L, Uppsala Univ, BMC, Immunol Programme, Dept Cell & Mol Biol, POB 596, S-75124 Uppsala, Sweden. Uppsala Univ, BMC, Immunol Programme, Dept Cell & Mol Biol, S-75124 Uppsala, Sweden.Available from: 2008-10-17 Created: 2008-10-17 Last updated: 2011-01-14 ...
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Genes encoding the different subunits of the IMMUNOGLOBULINS, for example the IMMUNOGLOBULIN LIGHT CHAIN GENES and the IMMUNOGLOBULIN HEAVY CHAIN GENES. The heavy and light immunoglobulin genes are present as gene segments in the germline cells. The completed genes are created when the segments are shuffled and assembled (B-LYMPHOCYTE GENE REARRANGEMENT) during B-LYMPHOCYTE maturation. The gene segments of the human light and heavy chain germline genes are symbolized V (variable), J (joining) and C (constant). The heavy chain germline genes have an additional segment D (diversity ...
Dive into the research topics of Immunohistologically definable light chain restriction in autoimmune disease: Authors reply. Together they form a unique fingerprint. ...
Disease response was evaluated according to the IMWG-URC using a validated computer algorithm. Overall response rate was defined as the percentage of participants with a best overall response of stringent complete response (sCR), complete response (CR), very good partial response (VGPR), or partial response (PR). sCR: As for CR, normal serum free light chain (SFLC) ratio and no clonal cells in bone marrow (BM). CR: No immunofixation on serum and urine, disappearance of any soft tissue plasmacytomas and , 5% plasma cells in BM biopsy; VGPR: Serum and urine M-protein detectable by immunofixation but not electrophoresis or ≥ 90% reduction in serum M-protein with urine M-protein ,100 mg/24 hours. A ≥ 50% reduction in the size of soft tissue plasmacytomas if present at baseline. PR: ≥ 50% reduction of serum M-protein and reduction in urine M-protein by ≥ 90% or to , 200 mg/24 hours. A ≥ 50% reduction in the size of soft tissue plasmacytomas if present at baseline ...
Disease response was evaluated according to the IMWG-URC using a validated computer algorithm. Overall response rate was defined as the percentage of participants with a best overall response of stringent complete response (sCR), complete response (CR), very good partial response (VGPR), or partial response (PR).. sCR: As for CR, normal serum free light chain (SFLC) ratio and no clonal cells in bone marrow (BM).. CR: No immunofixation on serum and urine, disappearance of any soft tissue plasmacytomas and , 5% plasma cells in BM biopsy; VGPR: Serum and urine M-protein detectable by immunofixation but not electrophoresis or ≥ 90% reduction in serum M-protein with urine M-protein ,100 mg/24 hours. A ≥ 50% reduction in the size of soft tissue plasmacytomas if present at baseline.. PR: ≥ 50% reduction of serum M-protein and reduction in urine M-protein by ≥ 90% or to , 200 mg/24 hours. A ≥ 50% reduction in the size of soft tissue plasmacytomas if present at baseline.. ...
Sakato, N; Semma, M; Eisen, H N.; and Azuma, T, A small hypervariable segment in the variable domain of an immunoglobulin light chain stimulates formation of anti-idiotypic suppressor t cells. (1982). Subject Strain Bibliography 1982. 3493 ...
This test looks for signs of antibodies called immunoglobulins in your blood. It can help diagnose an illness called multiple myeloma.
Northwestern Medicine offers a wide range of classes, events and support groups throughout the year - in your local community - to help you live a healthier, happier and more informed life.. ...
(HealthDay)-Elevated levels of polyclonal serum immunoglobulin combined free light chains (cFLCs) may indicate adverse cardiovascular disease (CVD) outcomes in patients with type 2 diabetes, according to research published ...
tetanospasmin: composed of a heavy chain and a light chain; the L chain is a zinc-dependent endopeptidase; classified as EC 3.4.24.68
Immunoglobulin light chain amyloidosis (AL) is a plasma cell dyscrasia characterized by deposition of amyloid fibrils in various organs and tissues, derived from monoclonal light chains, leading to organ dysfunction.1-3 High-dose melphalan with autologous stem cell transplant (HDM/SCT) is an effective treatment with high complete hematologic response rates (CR) and is capable of producing durable remissions and prolonged overall survival.4-6 Only selected patients are eligible to receive HDM/SCT, and treatment-related mortality is in the range of 5-15%. More effective and widely applicable treatment modalities in AL amyloidosis are, therefore, needed.. Clinical trials of alternate treatment options have tested non-transplant melphalan-based strategies and novel therapeutics such as lenalidomide and bortezomib. Oral melphalan and dexamethasone (M-Dex) is a standard regimen for patients not eligible to receive HDM/SCT; reported complete response rates range from 13% to 33% and median overall ...
TY - JOUR. T1 - Kappa light chain-associated Fanconis syndrome. T2 - Molecular analysis of monoclonal immunoglobulin light chains from patients with and without intracellular crystals. AU - Déret, Sophie. AU - Denoroy, Luc. AU - Lamarine, Marc. AU - Vidal, Ruben. AU - Mougenot, Béatrice. AU - Frangione, Blas. AU - Stevens, Fred J.. AU - Ronco, Pierre M.. AU - Aucouturier, Pierre. PY - 1999/5/17. Y1 - 1999/5/17. N2 - Plasma cell dyscrasias may be responsible for Fanconis syndrome, due to the toxicity of a free monoclonal kappa light chain toward kidney proximal tubules. Eight cases of Fanconis syndrome were analyzed. We compared the structures of VκI variability subgroup V domains from five cases of Fanconis syndrome and one myeloma without renal involvement. Among Fanconi cases, four putative structures were obtained after molecular modeling by homology, and the other had previously been refined by X-ray crystallography. The complete sequences of one VκI, one VκIII and N-terminal ...
Quock T, Yan T, Chang E et al. Epidemiology of AL amyloidosis: a real-world study using US claims data. Blood advances 2018; 2(10): 1046-1053. Kourelis T, Kumar S, Gertz M et al. Coexistent Multiple Myeloma or Increased Bone Marrow Plasma Cells Define Equally High-Risk POPULATINS in Patients With Immunoglobulin Light Chain Amyloidosis. Journal of clinical oncology 2013; 31(34): 4319-4324. Rafae A, Malik M N, Abu Zar M et al. An Overview of Light Chain Multiple Myeloma: Clinical Characteristics and Rarities, Management Strategies and Disease Monitoring. Cureus 2018; 10(8): 3148. Leung N, Bridoux F, Batuman V et al. The evaluation of monoclonal gammopathy of renal significance: a consensus report of the International Kidney and Monoclonal Gammopathy Research Group. Nature Reviews Nephrology 2019; 15: 45-59. Bowen K, Shah N, Lewin M. AL Amyloidosis Presenting with Negative Congo Red Staining in the Setting of High Clinical Suspicion: A Case Report. Case Reports in Nephrology 2012; 2012 Article ID ...
Abstract. Background: Primary systemic amyloidosis (AL) is an incurable plasma cell disorder. Lenalidomide, especially in conjunction with dexamethasone, has b
Results The direct numeric result from hs-cTnT measurement cannot merely be substituted for a cTnT measurement in the Mayo AL staging system. The performance of the receiver operator curve derived an hs-cTnT cut-point of 54 ng/L which improves on the value of 35 ng/L validated with the prior iteration of the assay. An alternate staging option using hs-cTnT alone-using the two thresholds 14 ng/L and 54 ng/L-performs as well as either the original Mayo AL staging system or other systems incorporating hs-cTnT. On multivariate analysis, an hs-cTnT alone staging system was independent of period of diagnosis, type of therapy, and NT-proBNP value, the last of which dropped out of the model. Alternate models were explored, but none performed better than the original system or the new hs-cTnT system. Thus, hs-cTnT can be used alone for the staging of disease prognosis.. ...
So why do I think all of this is predatory in nature? I receive this bill every 20 days, the same bill, and my husband and I make the required phone calls to the medical facility and the primary insurance company. So far neither institution has been able to resolve this claim. If we failed to make these phone calls every 20 days this bill, in all likelihood, would go to collection and could balloon to twice what it is now with fees and interest. That being said, in this case the medical facility actually owns the collection agency that these bills go to so they not only get to collect the amount of the bill, but the fees and interest as well. That doesnt sound shady at all does it?. What most people dont know or dont care to take advantage of is that if you call the medical facility that has sent you a bill to make payment arrangements or to dispute the charges they, in most circumstances, will not send you to collection; however, you have to call them EVERY TIME you receive the same bill. ...
TY - JOUR. T1 - Hereditary systemic immunoglobulin light-chain amyloidosis. AU - Benson, Merrill. AU - Liepnieks, Juris J.. AU - Kluve-Beckerman, Barbara. PY - 2015. Y1 - 2015. N2 - Several members of a family died from renal failure as a result of systemic amyloidosis. Extensive studies to detect previously documented gene mutations associated with amyloidosis failed to identify a causative factor. In search of the genetic basis for this syndrome, amyloid fibrils were isolated from renal tissue of a member of the kin who died while on renal dialysis. Amino acid sequencing of isolated amyloid protein identified sequences compatible with the constant region of the immunoglobulin κ light-chain. Isolation and characterization of κ light-chain protein from serum of an affected member of the kindred revealed mutation in the constant region of kκlight-chain, with cysteine replacing serine at amino acid residue 131. This mutation (Ser131Cys) was confirmed by DNA analysis, which identified a ...
Light chain deposition disease (LCDD) is a rare clinical disorder. The deposition of light chain immunoglobulins mainly affects the kidneys, which have different characteristics than other tissues. To date, the therapeutic approach for the treatment of LCDD has no evidence-based consensus, and clinical experience of reported cases guides current disease management strategies. The present systematic review investigates and summarizes the pathological mechanisms of renal injury and the subsequent treatments for LCDD.
TY - JOUR. T1 - Cardiac Light Chain Amyloidosis: The Role of Metal Ions in Oxidative Stress and Mitochondrial Damage. AU - Diomede, L.. AU - Romeo, M.. AU - Rognoni, P.. AU - Beeg, M.. AU - Foray, C.. AU - Ghibaudi, E.. AU - Palladini, G.. AU - Cherny, R. A.. AU - Verga, L.. AU - Capello, G. L.. AU - Perfetti, V.. AU - Fiordaliso, F.. AU - Merlini, G.. AU - Salmona, M.. N1 - LR: 20170920; JID: 100888899; OTO: NOTNLM; PMCR: 2018/09/20 00:00; 2018/09/20 00:00 [pmc-release]; 2017/01/31 06:00 [pubmed]; 2017/01/31 06:00 [medline]; 2017/01/31 06:00 [entrez]; ppublish. PY - 2017/9/20. Y1 - 2017/9/20. N2 - AIMS: The knowledge of the mechanism underlying the cardiac damage in immunoglobulin light chain (LC) amyloidosis (AL) is essential to develop novel therapies and improve patients outcome. Although an active role of reactive oxygen species (ROS) in LC-induced cardiotoxicity has already been envisaged, the actual mechanisms behind their generation remain elusive. This study was aimed at further ...
Current criteria for differential diagnosis of multiple myeloma (MM), Monoclonal gammopathy of undetermined significance (MGUS), and smoldering multiple myeloma (SMM) are included in the 2003 guidelines by the International Myeloma Working Group (IMWG). An updated version was then published in 2014, highlighting the importance of serum free light chain (sFLC) detection, as well as the κ/λ ratio as excellent indicators of clonality. At present, two commercial assays for sFLC quantification are available: the Freelite™ assay and the N-Latex assay. The first was developed by The Binding Site based on a mixture of polyclonal antibodies directed against a variety of FLC epitopes. It may be run on a wide range of nephelometers, as well as on turbidimeters. The second method was developed by Siemens and runs exclusively on Siemens instruments. It employs a probe mixture of mouse monoclonal antibodies. The aim of our study was to evaluate sFLC measurement and calculated κ/λ ratio in 85 patients with
Light chain amyloidosis, a deadly protein misfolding disease, is caused by multiple mutations in cells that are intended to protect the body. Instead, the mutations send misfolded bundles of proteins through the bloodstream, potentially destroying the heart, kidneys, liver or other organs. Mayo Clinic researchers have identified one of these mutations and have shown that the molecules shifting position is as important as its unique shape. The findings appear in the current issue of the journal Structure.
OBJECTIVES: This study investigated the prevalence and potential incremental prognostic value of combined free light chains (cFLCs) in patients recently hospitalized with decompensated heart failure (HF). BACKGROUND: Inflammatory pathways are recognized in the pathogenesis and progression of HF. Free light chain (FLC) elevation is conventionally associated with monoclonal gammopathies, including multiple myeloma. Polyclonal increases in both kappa and lambda FLCs occur in autoimmune and other chronic inflammatory conditions. Recently, a novel assay for measuring kappa and lambda immunoglobulin FLCs together, known as combined free light chain (cFLC) has been developed. METHODS: Six hundred twenty-eight patients recently hospitalized with decompensated HF were studied. cFLCs were measured by turbidimetry using an immunoassay. The incremental prognostic value of cFLCs for mortality was evaluated using Cox proportional hazard models including 22 established predictors of outcome in HF. RESULTS: Of 628
Lenti ORF particles, Igk (Myc-DDK-tagged) - Mouse immunoglobulin kappa chain, constant region (cDNA clone MGC:102611 IMAGE:4219669), 200 uL, |10^7 TU/mL, 200 µl.
Cardiovascular diseases remain a significant cause of morbidity and mortality in developed countries. The amyloidoses are an important group of systemic diseases that cause substantial cardiac impairment. Recently there have been advances in the manner in which these diseases are diagnosed, classified, and treated. Topics to be covered in this session included amyloid subtyping techniques including mass spectrometry, classification of the amyloidoses including new and rare forms of amyloid, the state of the art use of protein folding inhibitors to treat amyloidosis, and the results obtained from an a new international consortium for performing cardiac transplantation coupled with stem cell transplantation to treat immunoglobulin light chain amyloidosis. The session will be capped by Dr. Michael Fishbein, the recipient of the SCVP Distinguished Achievement Award, speaking on arteriosclerosis. In addition, there will be a portion of the session devoted to short presentations by a selected subset ...
Sjögren’s syndrome, sarcoidosis and systemic immunoglobulin light chain amyloidosis all share common features when analyzed through salivary gland ultrasound, according to findings published in Arthritis Care & Research.“Ultrasound is becoming substantially more popular for imaging of salivary glands in the rheumatology community,” Eugene Kissin, MD, of Boston University
A common renal complication of multiple myeloma is myeloma kidney, a condition also known as cast nephropathy. The renal lesions (casts) are directly related to the production of monoclonal immunoglobulin free light chains (FLCs), which coprecipitate with Tamm-Horsfall glycoprotein (THP) in the lumen of the distal nephron, obstructing tubular fluid flow. Here, we report that analysis of the binding interaction between FLCs and THP demonstrates that the secondary structure and key amino acid residues on the complementarity-determining region 3 (CDR3) of FLCs are critically important determinants of the molecular interaction with THP. The findings permitted development of a cyclized competitor peptide that demonstrated strong inhibitory capability in the binding of FLCs to THP in vitro. When used in a rodent model of cast nephropathy, this cyclized peptide construct served as an effective inhibitor of intraluminal cast formation and prevented the functional manifestations of acute kidney injury ...
HCAK 1, HCAK1, Ig kappa chain C region, IGKC, Immunoglobulin kappa constant, Immunoglobulin kappa constant region, Immunoglobulin kappa light chain, Kappa 1 immunoglobulin light chain, Km, MGC111575, MGC62011, MGC72072, MGC88770, MGC88771, MGC88809.
The monoclonal antibody, MDX-1097, is currently progressing through clinical trials as a possible therapy for multiple myeloma. MDX-1097 targets a cell membrane bound form of free immunoglobulin kappa light chain (FkappaLC), termed kappa myeloma antigen (KMA), which is found on the surface of malignant plasma cells. The clinical potential of MDX-1097 highlights the need to characterise the expression of its cognate antigen, KMA, in normal tissue. In this study, we have analysed the expression of KMA on B cell subsets found in tonsils, peripheral blood and bone marrow. We found KMA expression on a small population of tonsillar and in vitro derived plasmablasts. In contrast, no KMA expression was observed on peripheral blood or bone marrow resident B cell subsets. This study yields important insights into the possible subsets of B cells that might be depleted as a result of an immunotherapy targeting KMA.
Rat anti Mouse kappa light chain antibody, clone LO-MK-1 recognises the murine kappa immunoglobulin light chain, and does not cross-react
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Rabbit light chain 3315, prepared from a homogeneous antipneumococcal antibody, was subjected to hydrolysis by pepsin without prior reduction and alkylation of the intrachain disulfide bonds. Gel filtration of the hydrolysate on Sephadex G-10, G-15, and G-25 and ion exchange chromatography on SP-Sephadex yielded several disulfide bridge peptides. These were fully reduced and alkylated and sequenced by Edman degradation. The peptides were located in the light chain sequence determined in independent studies from our laboratory.. The half-cystine residues in this κB rabbit chain are located at positions 23, 80, 88, 134, 171, 194, and 214. The extra disulfide bridge extends between residues 80 and 171, thus joining the variable and constant domains. This is consistent with x-ray diffraction crystallographic studies showing that the corresponding residues in human light chains are separated by a distance compatible with disulfide bond formation.. ...
Gentaur molecular products has all kinds of products like :search , AbD \ GOAT ANTI MOUSE LAMBDA LIGHT CHAIN Alk. Phos., Product Type Polyclonal Antibody, Specificity LAMBDA LIGHT CHAIN, Target Species Mouse, Host Goat, Format Alk. Phos., Isotypes Polyclonal IgG, Appl \ 106004 for more molecular products just contact us
Hozumi, N; Wu, G; Murialdo, H; Baumal, R; Mosmann, T; Winberry, L; and Marks, A, Arrangement of lambda light chain genes in mutant clones of the mopc 315 mouse myeloma cells. (1982). Subject Strain Bibliography 1982. 402 ...
Pathogenic antibodies or antibody fragments. Normal antibodies are composed of two heavy chains encoded by the IgH locus and two light chains, which may be encoded by either the Ig kappa or the Ig lambda locus. Multiple myeloma cells most commonly secrete IgG or IgA antibodies. However, in addition to complete antibodies, neoplastic plasma cells usually also secrete free, unpaired Ig light chains; indeed, in about 20% of cases, only light chains are secreted. The small size of the light chains (around 25 kDa) permits them to pass from the blood through the filtration slits of the renal glomeruli and into the renal tubules. Once in the urinary space, Ig light chains are toxic to renal epithelial cells and tend to form precipitates and obstructive casts, both of which contribute to renal dysfunction (Figure 24-1). Free light chains, particularly lambda light chains, also are prone to form amyloid, fibrillar deposits that may be found in the renal glomeruli (Figure 24-2) and the perivascular spaces ...
|strong|Mouse anti Human kappa light chain antibody, clone 4G7|/strong| recognizes a conformation-independent, constantly expressed epitope of the human immunoglobulin kappa chain.
Uphaus T, Bittner S, Groschel S, Steffen F, Muthuraman M, Wasser K, Weber-Kruger M, Zipp F, Wachter R and Groschel K. Stroke. 2019 Sep 20:STROKEAHA119026410. doi: 10.1161/STROKEAHA.119.026410
GENETICS IN MEDICINE | NOVEMBER 26, 2020 Dang Do AN, Sinaii N, Masvekar RR, Baker EH, Thurm AE, Soldatos AG, Bianconi SE, Bielekova B and Porter FD. Genet
Primary amyloidosis is a systemic disorder caused by the clonal production and tissue deposition of immunoglobulin light chain proteins. The disease symptoms are typical of multisystem failure. Common presenting features include nephrotic syndrome, hepatomegaly, sensomotor peripheral neuropathy and, in the case of cardiac involvement, congestive heart failure. This last sign appears very seldom as alone, without any others. Cardiac involvement generally denotes a poor prognosis, regardless of the method of treatment. The median survival rate from onset of congestive heart failure is 6 months. Only the patients with earliest diagnosis made and advanced treatment (chemotherapy, autologous stem-cell transplantation, heart transplantation) introduced have the chance of the lengthening of life. The authors present a case of 52-year-old man with a primary amyloidosis, who suffered from severe, not responding to treatment, congestive heart failure. Because of lack of the other organ involvement ...
Mouse monoclonal Lambda Free Light Chain antibody [3D12] validated for WB, ELISA, IHC, Other and tested in Human. With 1 independent review. Immunogen…
Lambda Light Chain, 1 mg. |p class=db_paraghraph|Lambda light chain is the smaller of the two types of polypeptide chains in immunoglobulins, consisting of an antigen-binding segment with a variable amino acid sequence, and a constant region with a
AL Amyloidosis is known to be a systemic disease affecting multiple organs and tissue while its rare that patients present with gastrointestinal symptoms at first and later develop multiple-organ dysfuction. Clinical signs are not specific and the diagnosis is rarely given before performing immunofixation and endoscopy with multiple biopsies. We would like to emphasize the value of precise diagnostic process of AL amyloidosis. In this case report, we describe a 56-year-old man who presented with recurrent periumbilical pain for 4 months and gradually worsened over a month. After a series of tests, he was finally diagnosed with primary systemic AL amyloidosis. He was treated with a chemotherapy regimen (Melphalan, dexamethasone and thalidomide) achieving a good clinical response. On account of the high misdiagnosis rate, establishing the most precise diagnosis in first time with typing amyloidogenic protein becomes increasingly vital. Although the presenting feature is usually nonspecific, AL
V region of the variable domain of immunoglobulin light chains that participates in the antigen recognition. Immunoglobulins, also known as antibodies, are membrane-bound or secreted glycoproteins produced by B lymphocytes. In the recognition phase of humoral immunity, the membrane-bound immunoglobulins serve as receptors which, upon binding of a specific antigen, trigger the clonal expansion and differentiation of B lymphocytes into immunoglobulins-secreting plasma cells. Secreted immunoglobulins mediate the effector phase of humoral immunity, which results in the elimination of bound antigens (PubMed:20176268, PubMed:22158414). The antigen binding site is formed by the variable domain of one heavy chain, together with that of its associated light chain. Thus, each immunoglobulin has two antigen binding sites with remarkable affinity for a particular antigen. The variable domains are assembled by a process called V-(D)-J rearrangement and can then be subjected to somatic hypermutations which, ...
V segment of the variable domain of immunoglobulins light chain that participates to the antigen recognition (PubMed:24600447). Immunoglobulins, also known as antibodies, are membrane-bound or secreted glycoproteins produced by B lymphocytes. In the recognition phase of humoral immunity, the membrane-bound immunoglobulins serve as receptors which, upon binding of a specific antigen, trigger the clonal expansion and differentiation of B lymphocytes into immunoglobulins-secreting plasma cells. Secreted immunoglobulins mediate the effector phase of humoral immunity, which results in the elimination of bound antigens (PubMed:22158414, PubMed:20176268). The antigen binding site is formed by the variable domain of one heavy chain, together with that of its associated light chain. Thus, each immunoglobulin has two antigen binding sites with remarkable affinity for a particular antigen. The variable domains are assembled by a process called V-(D)-J rearrangement and can then be subjected to somatic
Supplementary MaterialsSupplementary Information 41467_2019_9133_MOESM1_ESM. composed of an individual protofilament, showing usual 4.9?? stacking and combination- structures. Two distinctive polypeptide exercises (total of 77 residues) from your LC variable website (Vl) match the fibril denseness. Despite Vl high sequence variability, residues stabilizing the fibril core are conserved through different cardiotoxic Vl, highlighting structural motifs that may be common to misfolding-prone LCs. Our data shed light on the architecture of LC amyloids, correlate amino acid sequences with fibril assembly, providing the grounds for development of innovative medicines. Introduction Light chain amyloidosis?(AL), with an incidence of about 10 new instances per million-persons/year, is currently the most TMC353121 common systemic form of amyloidosis in Western countries1. The disease is definitely associated with the presence of a plasma cell clone, and is caused by extracellular deposition of ...
Anti-CD38 Antibodies for Treatment of Light Chain Amyloidosis and Other CD28-Positive Hematological Malignancies | ANTI-PROLACTIN RECEPTOR ANTIBODY FORMULATIONS | ANTIBODY DRUG CONJUGATES | TREATMENT OF CANCER USING HUMANIZED ANTI-EGFRvIII CHIMERIC ANTIGEN RECEPTOR | THERAPEUTIC PEPTIDES |
Biopsy, Cell, Disease, Immunoglobulin, Immunoglobulin Light Chains, Kidney, Kidney Disease, Knowledge, Light, Lymphoma, Patients, Plasma, Plasma Cell, Plasma Cell Dyscrasias, Production, Report, Set, Therapeutic
The purpose of this study is to evaluate the predictive role of free Kappa/Lambda ration in Multiple Myeloma post-transplant.
LILRA2 [ENSP00000251377]. Leukocyte immunoglobulin-like receptor, subfamily A (with TM domain), member 2; Part of the innate immune responses against microbial infection. Specifically recognizes a set of N-terminally truncated immunoglobulins that are produced via cleavage by proteases from a range of pathogenic bacteria and fungi, including L.pneumophila, M.hyorhinis, S.pneumoniae, S.aureus and C.albicans. Recognizes epitopes that are in part in the variable region of the immunoglobulin light chains, but requires also the constant region for signaling. Binds to a subset of cleaved IgM, IgG3 and IgG4 molecules, but does not bind cleaved IgA1. Binding of N-terminally truncated immunoglobulins mediates activation of neutrophils. In monocytes, activation leads to the release of CSF2, CF3, IL6, CXCL8 and CCL3 and down-regulates responses to bacterial lipopolysaccharide (LPS), possibly via down-regulation of TLR4 expression and reduced signaling via TLR4. In eosinophils, activation by ligand binding ...
There are no specific protocols for Rat monoclonal JC5-1 Anti-Mouse lambda light chain (Alkaline Phosphatase) (ab99624). Please download our general protocols…
"Systemic immunoglobulin light chain amyloidosis" (PDF). Nature Reviews. Disease Primers. 4 (1): 38. doi:10.1038/s41572-018-0034 ... Amyloid light chains deposition in shoulder joint causes enlarged shoulders, also known as "shoulder pad sign". Amyloid light ... such as with overproduction of immunoglobulin light chains (termed AL amyloidosis), or with continuous overproduction of acute ... Falk RH, Alexander KM, Liao R, Dorbala S (September 2016). "AL (Light-Chain) Cardiac Amyloidosis: A Review of Diagnosis and ...
The key observation by César Milstein and colleagues was that immunoglobulin light chains were produced in a higher molecular ... Milstein C, Brownlee GG, Harrison TM, Mathews MB (September 1972). "A possible precursor of immunoglobulin light chains". ... I. Presence of proteolytically processed and unprocessed nascent immunoglobulin light chains on membrane-bound ribosomes of ...
I. Presence of proteolytically processed and unprocessed nascent immunoglobulin light chains on membrane-bound ribosomes of ... particularly immunoglobulin light chains; and bovine preprolactin. Newly synthesized proteins in eukaryotes carry N-terminal ... "A possible precursor of immunoglobulin light chains". Nature. 239 (91): 117-20. doi:10.1038/newbio239117a0. PMID 4507519. ... SRP then targets this entire complex (the ribosome-nascent chain complex) to the protein-conducting channel, also known as the ...
Binding of mouse immunoglobulins is restricted to those having VκI light chains. Given these specific requirements for ... In humans and mice, most antibody molecules contain kappa (κ) light chains and the remainder have lambda (λ) light chains. ... Akerström B, Björck L (November 1989). "Protein L: an immunoglobulin light chain-binding bacterial protein. Characterization of ... were found to be responsible for the interaction with Ig light chains.[2] In addition to Protein L, other immunoglobulin- ...
September 2006). "Immunoglobulin free light chains and solitary plasmacytoma of bone". Blood. 108 (6): 1979-83. doi:10.1182/ ...
... of immunoglobulin (Ig) heavy chain and light chain genes in B cells forms the genetic basis for the presence ... Pelanda R (April 2014). "Dual immunoglobulin light chain B cells: Trojan horses of autoimmunity?". Current Opinion in ... The allelic exclusion of light chain genes Igκ and Igλ is a process that is controlled by the monoallelic initiation of V(D)J ... "Immunoglobulin Heavy Chain Variable, Diversity, and Joining Region Gene Rearrangement". National Cancer Institute Thesaurus. ...
Badar T, D'Souza A, Hari P (2018). "Recent advances in understanding and treating immunoglobulin light chain amyloidosis". ... In immunoglobulin light chain amyloidosis (AL amyloidosis), chemotherapy can be used to lower the number of the blood cells ... that make the light chain protein that forms amyloid in various bodily organs. Transthyretin (TTR) amyloidosis (ATTR) results ... and that all amyloids show birefringence in cross-polarized light after staining with the dye Congo red, as well as a fibrillar ...
For light chain amyloidosis, serum immunoglobulin free light chain assay can be used for diagnosis and following of the ... Pathogenesis of this form is due to the aggregation of immunoglobulin lambda light chains. These chains are created by an ... Light chain (AL-CM) Prognosis: For light-chain amyloidosis early detection leads to best possibility of therapies prolonging ... the major goal of treatment is the reduction in concentration of light chains. For light-chain amyloidosis, the use of FLC ...
Gertz MA (December 2014). "Immunoglobulin light chain amyloidosis: 2014 update on diagnosis, prognosis, and treatment". ...
The light chains can be immunoglobulin fragments or single homogeneous immunoglobulins. They are found in urine as a result of ... The proteins are immunoglobulin light chains (paraproteins) and are produced by neoplastic plasma cells. They can be kappa ( ... A Bence Jones protein is a monoclonal globulin protein or immunoglobulin light chain found in the urine, with a molecular ... "Outcome in systemic AL amyloidosis in relation to changes in concentration of circulating free immunoglobulin light chains ...
Kenny TP, Semrad TJ, Malyj W, Robbins DL (2001). "Mutational analysis of immunoglobulin germline derived Vlambda4B light chains ... Wang X, Stollar BD (1999). "Immunoglobulin VH gene expression in human aging". Clin. Immunol. 93 (2): 132-42. doi:10.1006/clim. ...
These plasmablasts express IgM-immunoglobulin light chains, most often of lambda subtype. These plasmablasts can give rise to a ... It has variable CD20 expression and unmutated immunoglobulin variable region genes. Castleman disease (CD) is a ... The plasmablasts do not show rearranged immunoglobulin genes, and typically lack EBV infection. The disease predominantly ...
Both alpha and beta chains share significant homology to immunoglobulin variable light chains. This gene encodes the CD8 alpha ... The functional coreceptor is either a homodimer composed of two alpha chains, or a heterodimer composed of one alpha and one ... Nakayama K, Tokito S, Okumura K, Nakauchi H (1989). "Structure and expression of the gene encoding CD8 alpha chain (Leu-2/T8 ... Human T-cell surface glycoprotein CD8 alpha chain) at the PDBe-KB. Overview of all the structural information available in the ...
Clonal rearrangements of the immunoglobulin genes (heavy and light chains) are frequently seen. The deletion 7q21-32 is seen in ... Dunn-Walters DK, Boursier L, Spencer J, Isaacson PG (June 1998). "Analysis of immunoglobulin genes in splenic marginal zone ...
Modified proteins such as immunoglobulin light chains abnormally accumulate between cells, forming fibrils. Multiple folds of ... Similarly, light modulators modulate the intensity of light through electrically induced birefringence of polarized light ... Unpolarized light consists of equal amounts of energy in any two orthogonal polarizations, and even polarized light (except in ... Light from the source is polarized in the x direction after passing through the first polarizer, but above the specimen is a ...
Each immunoglobulin molecule consists of two identical heavy chains and two identical light chains. There are two classes of ... Combriato G, Klobeck HG (1991). "V lambda and J lambda-C lambda gene segments of the human immunoglobulin lambda light chain ... Hieter PA, Korsmeyer SJ, Waldmann TA, Leder P (1981). "Human immunoglobulin kappa light-chain genes are deleted or rearranged ... 1982). "Chromosomal location of human kappa and lambda immunoglobulin light chain constant region genes". J. Exp. Med. 155 (5 ...
They are formed by light and heavy chain of the variable region of an immunoglobulin. The two chains are linked by a flexible ... They mostly consist of a heavy and light chain of the variable region of immunoglobulin. Recombinant antibodies have many ... "Assembly of functional antibodies from immunoglobulin heavy and light chains synthesised in E. coli". Nucleic Acids Research. ... day production of scFv was the successful assembly of recombinant antibodies from heavy and light chain of immunoglobulin. ...
"Variable regions of heavy and light polypeptide chains of the same gammaG-immunoglobulin molecule". Proceedings of the National ... the larger heavy chains and the smaller light chains. Two light and two heavy chains are linked together by disulfide bonds to ... V. Partial amino acid sequence of the light chain". Biochemistry. 7 (5): 1983-1994. doi:10.1021/bi00845a049. PMID 5650389. ... include amino acids from both the light and heavy protein subunits. The inter-chain disulfide bonds help bring together the two ...
Each immunoglobulin molecule consists of two identical heavy chains and two identical light chains. This region represents the ... immunoglobulin genes: Heavy chain alpha (IgA): IGHA1, IGHA2 Heavy chain gamma (IgG): IGHG1, IGHG2, IGHG3, IGHG4 Heavy chain ... Immunoglobulin heavy locus, also known as IGH, is a region on human chromosome 14 that contains a gene for the heavy chains of ... Buluwela L, Rabbitts TH (1989). "A VH gene is located within 95 Kb of the human immunoglobulin heavy chain constant region ...
"The covalent structure of a human gamma G-immunoglobulin. V. Partial amino acid sequence of the light chain". Biochemistry. 7 ( ... The protein subunits of antibodies are of two types, the larger heavy chains and the smaller light chains. Gottlieb's ... "Variable regions of heavy and light polypeptide chains of the same gammaG-immunoglobulin molecule". Proceedings of the National ... contribution was to determine a considerable part of the molecular structure of the light chains. He was a postdoc at ...
Bertocci B, De Smet A, Berek C, Weill JC, Reynaud CA (August 2003). "Immunoglobulin kappa light chain gene rearrangement is ... Whereas pol μ is important for heavy-chain rearrangements, pol λ seems to be more important for light-chain rearrangements. The ... and terminal deoxynucleotidyltransferase during immunoglobulin V(D)J recombination in vivo". Immunity. 25 (1): 31-41. doi: ...
Bertocci B, De Smet A, Berek C, Weill JC, Reynaud CA (August 2003). "Immunoglobulin kappa light chain gene rearrangement is ... but only during light chain rearrangements. This is distinct from pol λ, which is involved in heavy chain rearrangements. In ... and terminal deoxynucleotidyltransferase during immunoglobulin V(D)J recombination in vivo" (PDF). Immunity. 25 (1): 31-41. doi ...
"Structure of peptostreptococcal protein L and identification of a repeated immunoglobulin light chain-binding domain". J. Biol ... These repeats have been found to be responsible for the interaction of protein L with Ig light chains. N-terminus domain ... light chain-binding properties. It contains a number of homologous b1 repeats towards the N terminus. ... In molecular biology, the protein domain b1 refers to the domain b1 of Protein L. L is a bacterial protein with immunoglobulin ...
"Sequence of a mouse germ-line gene for a variable region of an immunoglobulin light chain". Proceedings of the National Academy ... "A tissue-specific transcription enhancer element is located in the major intron of a rearranged immunoglobulin heavy chain gene ... "Transcriptional enhancer elements in the mouse immunoglobulin heavy chain locus". Science. 221 (4611): 663-665. Bibcode:1983Sci ... Research based on the split gene theory sheds light on other basic questions of exons and introns. The exons of eukaryotes are ...
"Sequence of a mouse germ-line gene for a variable region of an immunoglobulin light chain". Proceedings of the National Academy ...
Immunoglobulin kappa constant, also known as IGKC, is a human gene that encodes the constant domain of kappa-type light chains ... Klobeck HG, Combriato G, Zachau HG (1984). "Immunoglobulin genes of the kappa light chain type from two human lymphoid cell ... 2001). "Both the environment and somatic mutations govern the aggregation pathway of pathogenic immunoglobulin light chain". J ... kappa IV immunoglobulin light chain". Biochem. Biophys. Res. Commun. 245 (3): 713-6. doi:10.1006/bbrc.1998.8515. PMID 9588180. ...
"Sequence of a mouse germ-line gene for a variable region of an immunoglobulin light chain". Proceedings of the National Academy ... possibly identifying species-specific biases that may shed light on varied rates of intron gain amongst different species. ...
"Inhibition by small-molecule ligands of formation of amyloid fibrils of an immunoglobulin light chain variable domain". eLife. ...
B-cells from LMP2A transgenic E line undergo immunoglobulin light chain rearrangements, but not heavy chain rearrangement. This ... indicates that LMP2A signaling bypasses the requirement for immunoglobulin recombination and allows immunoglobulin M-negative ...
Primary amyloidosis (AL) is caused by the deposition of excess immunoglobulin light chains which are secreted from plasma cells ... Plasma cells can only produce a single kind of antibody in a single class of immunoglobulin. In other words, every B cell is ... Plasma cells are large lymphocytes with a considerable nucleus-to-cytoplasm ratio and a characteristic appearance on light ... Educational Resource on the Biology of Immunoglobulins. *Histology image: 21001loa - Histology Learning System at Boston ...
In a 2002-2003 survey of 1,030 animals including 679 bats from Gabon and the Republic of the Congo, immunoglobulin G (IgG) ... "This is the first time that all three countries - Guinea, Liberia and Sierra Leone - have stopped the original chains of ... such as light sensitivity, excess tearing, and vision loss have been described.[142] ... detecting the viral RNA by polymerase chain reaction (PCR)[6][23] and detecting proteins by enzyme-linked immunosorbent assay ( ...
High titers of either immunoglobulin G (IgG) or immunoglobulin M (IgM) antibodies to Borrelia antigens indicate disease, but ... Polymerase chain reaction (PCR) tests for Lyme disease have also been developed to detect the genetic material (DNA) of the ... Light-colored clothing may make it easier to see ticks and remove them before they bite.[132] Military and outdoor workers' ... usually mild meningitis signs such as inability to flex the neck fully and intolerance to bright lights, but typically no or ...
Antibodies are typically made of basic structural units-each with two large heavy chains and two small light chains. There are ... Heavy chains[change , change source]. Antibodies are glycoproteins belonging to the immunoglobulin superfamily; the terms ... 4. Light chain (green) with one variable (VL) and one constant (CL) domain. 5. Antigen binding site (paratope). 6. Hinge ... Immunoglobulin diversity[change , change source]. Basic issue[change , change source]. Although a huge variety of different ...
Hyper-IgM syndromes Normal numbers of B cells with isotype or light chain deficiencies: heavy chain deletions, kappa chain ... immunoglobulin replacement therapy in the form of intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin (SCIG) may ... For instance, an antibody deficiency can be diagnosed in the presence of low immunoglobulins, recurrent infections and failure ... Milder forms of primary immunodeficiency, such as selective immunoglobulin A deficiency, are fairly common, with random groups ...
... resulting in different types of polyubiquitin chains.[54] Chains in which each additional ubiquitin is linked to lysine 48 of ... In this neutralisation pathway, TRIM21 (a protein of the tripartite motif family) binds with immunoglobulin G to direct the ... Light blue chemical structures are the inhibitor bortezomib bound to the active sites. ... The result is a polyubiquitin chain that is bound by the proteasome, allowing it to degrade the tagged protein.[1] The ...
food chain. The chain of eating and getting nutrition which starts from a small herbivores animal and ends up at a big ... light-independent reactions. See Calvin cycle.. linked genes. Any set of one or more genes which are sufficiently close ... immunoglobulin. Any of a class of glycoprotein molecules produced by plasma cells (white blood cells) which act as a critical ... A polypeptide chain of amino acids. It is a body-building nutrient.. protist. psychobiology. Also called behavioral ...
negative regulation of myosin-light-chain-phosphatase activity. • negative regulation of transcription, DNA-templated. • ... regulation of immunoglobulin secretion. • osteoclast differentiation. • regulation of tumor necrosis factor-mediated signaling ... 2007). "TNF Trafficking to Human Mast Cell Granules: Mature Chain-Dependent Endocytosis". The Journal of Immunology. 178 (9): ... Olszewski MB, Groot AJ, Dastych J, Knol EF (May 2007). "TNF trafficking to human mast cell granules: mature chain-dependent ...
... also called immunoglobulins). The TCR is similar to a half-antibody consisting of a single heavy and single light chain, except ... Each chain is composed of two extracellular domains: Variable (V) region and a Constant (C) region, both of Immunoglobulin ... The TCR alpha chain is generated by VJ recombination, whereas the beta chain is generated by VDJ recombination (both involving ... Likewise, generation of the TCR gamma chain involves VJ recombination, whereas generation of the TCR delta chain occurs by VDJ ...
They can, however, confirm an allergy in light of a patient's history of reactions to a particular food. Non-IgE-mediated ... From two reviews, maternal intake of omega-3, long-chain fatty acids during pregnancy appeared to reduce the risks of medically ... Non-IgE mediated - characterized by an immune response not involving immunoglobulin E; may occur some hours after eating, ... After many public protests, Sony Pictures and the director apologized for making light of food allergies.[92] ...
Monomers of IgE consist of two heavy chainschain) and two light chains, with the ε chain containing 4 Ig-like constant ... Immunoglobulin E (IgE) is a type of antibody (or immunoglobulin (Ig) "isotype") that has only been found in mammals. IgE is ... Presence of a unique immunoglobulin as a carrier of reaginic activity". J. Immunol. 97 (1): 75-85. PMID 4162440.. ... Winter WE, Hardt NS, Fuhrman S (2000). "Immunoglobulin E: importance in parasitic infections and hypersensitivity responses". ...
In Eastern Christianity it is common to hang decorated common ostrich eggs on the chains holding the oil lamps.[92] The initial ... Their skin varies in colour depending on the subspecies, with some having light or dark grey skin and others having pinkish or ... This is in contrast to domesticated ostriches, who in captivity develop high concentration of immunoglobulin antibodies in ... total short-chain fatty acids (SCFA), and acetate.[75] The caecum contains a high water concentration with reduced levels ...
The amino acids in a polypeptide chain are linked by peptide bonds. Once linked in the protein chain, an individual amino acid ... As proteins are too small to be seen under a light microscope, other methods have to be employed to determine their structure. ... From left to right are: immunoglobulin G (IgG, an antibody), hemoglobin, insulin (a hormone), adenylate kinase (an enzyme), and ... A linear chain of amino acid residues is called a polypeptide. A protein contains at least one long polypeptide. Short ...
... inglise light chains). Raske ahel koosneb neljast eri geeniosade kodeeritud valgudomeenist, mida tähistatakse (N-otsast lugedes ... J Wang, D Lin, H Peng, Y Huang, J Huang ja J Gu, Cancer-derived immunoglobulin G promotes tumor cell growth and proliferation ... Kaht suuremat ja omavahel identset ahelat nimetatakse rasketeks ehk H-ahelateks (ingl.k heavy chains), kaht väiksemat ning ... Roald Nezlin, The Immunoglobulins: Structure and Function, lk 93, 1998, Academic Press, Google'i raamat veebiversioon (vaadatud ...
Furthermore, Streptococcus dysgalactiae possesses Protein G, a virulence factor binding circulating immunoglobulins, and thus ... Streptococcus meaning chain forming (Streptos) rounded berry-like bodies (kokkos), referring to their usual appearance under a ... light-microscope. Dys (bad) galactiae (milk) alludes to their propensity to cause bovine mastitis. Equi (horse) similis (like) ...
... next to the immunoglobulin heavy- or light-chain gene enhancers, leading to increased C-MYC expression and increased cell ... The cDNA is sequenced and the sequence encoding the variable heavy and variable light chains of these antibodies are cloned ... This technology uses a single chain variable fragment (scFv) designed to recognize the cell surface marker CD19 as a method of ...
Her death would indirectly spark a chain of events that would eventually lead to the permanent ousting of the Stuart line from ... Serologic tests and enzyme linked immunosorbent assays (ELISA), which measured Variola virus-specific immunoglobulin and ... Afforded scientific credibility through the studies by Niels Ryberg Finsen showing that red light reduced scarring,[3] this ... Strains were characterized by polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) analysis. ...
Single polymer chains (0.4 nm thick) recorded in a tapping mode under aqueous media with different pH.[10] ... In this method, laser light from a solid-state diode is reflected off the back of the cantilever and collected by a position- ... Rief, M; Gautel, M; Oesterhelt, F; Fernandez, J M; Gaub, H E (1997). "Reversible Unfolding of Individual Titin Immunoglobulin ... Angular displacement of the cantilever results in one photodiode collecting more light than the other photodiode, producing an ...
An antibody is made up of two heavy chains and two light chains. The unique variable region allows an antibody to recognize its ... Antibodies (also known as immunoglobulin, Ig), are large Y-shaped proteins used by the immune system to identify and neutralize ... In antibody responses, B cells are activated to secrete antibodies, which are proteins also known as immunoglobulins. ... "AgDscam, a hypervariable immunoglobulin domain-containing receptor of the Anopheles gambiae innate immune system". PLOS ...
Light chain[edit]. Further information: Immunoglobulin light chain. In mammals there are two types of immunoglobulin light ... Several immunoglobulin domains make up the two heavy chains (red and blue) and the two light chains (green and yellow) of an ... Heavy chain[edit]. Further information: Immunoglobulin heavy chain. There are five types of mammalian Ig heavy chain denoted by ... The approximate length of a light chain is 211 to 217 amino acids.[2] Each antibody contains two light chains that are always ...
Gertz (Dec 2014). "Immunoglobulin light chain amyloidosis: 2014 update on diagnosis, prognosis, and treatment". Am J Hematol. ...
Intravenous immunoglobulins (IVIGs). Intravenous immunoglobulins may be used to control SLE with organ involvement, or ... Each protein is a link on the autoimmune chain, and researchers are trying to find drugs to break each of those links.[10][56][ ... In the case of clearance deficiency, apoptotic nuclear debris accumulates in the light zone of GC and gets attached to FDC. ... Rhodes B, Vyse TJ (November 2008). "The genetics of SLE: an update in the light of genome-wide association studies". ...
... two regulatory light chains (20 KDa) controlling the myosin activity, and two essential light chains (17 kDa), which stabilize ... Plasma immunoglobulins. Normal Haematology. Normal Peripheral blood lymphocytes. Normal Micronucleus test. Normal ... Myosin-9 also known as myosin, heavy chain 9, non-muscle or non-muscle myosin heavy chain IIa (NMMHC-IIA) is a protein which in ... All three paralogs appear to bind the same or very similar light chains and share basic properties as to structure and ...
Between November and February, cubs are born blind, covered with a light down fur, and weighing less than 0.9 kg (2.0 lb),[118] ... and biomarkers such as immunoglobulin G and retinol suggest similar effects on polar bears. PCBs have received the most study, ... in polar bear tissues continued to rise for decades after being banned as these chemicals spread through the food chain. Since ... The hollow guard hairs of a polar bear coat were once thought to act as fiber-optic tubes to conduct light to its black skin, ...
Milstein C, Brownlee GG, Harrison TM, Mathews MB (1972). "A possible precursor of immunoglobulin light chains". Nature: New ... I. Presence of proteolytically processed and unprocessed nascent immunoglobulin light chains on membrane-bound ribosomes of ...
Jeed Antikörper is ut twee glieke swore Keden (heavy chains, H) un twee glieke lichte Keden (light chains, L) tohopensett, de ... Immunoglobulin W[ännern , Bornkood ännern]. Immunglobulin W (IgW) is eerst 1996 bi en Oort vun de Haien opdeckt worrn. Ut ... Alexander H. Lucas (2001): Immunoglobulin Gene Construction. In: Encyclopedia of Life Sciences. doi:10.1038/npg.els.000117 ( ...
For his researches connected with the wave theory of light, contained in the Transactions of the Royal Irish Academy ... In recognition of his elucidation of the structure of immunoglobulins and of the reactions involved in activating the ... In recognition of his seminal contributions to understanding the fundamental dynamics of carbon chain molecules, leading to the ... On account of his curious Experiments and Discoveries concerning the different refrangibility of the Rays of Light, ...
New Criteria for Response to Treatment in Immunoglobulin Light Chain Amyloidosis Based on Free Light Chain Measurement and ... AL amiloidozė (angl. amyloid light chain), yra dažniausiai pasitaikanti amiloidozės forma, kuriai būdinga limfocitų B ląstelių ... Evaluation of the cytogenetic aberration pattern in amyloid light chain amyloidosis as compared to monoclonal gammopathy of ... Bortezomib with or without dexamethasone in primary systemic (light chain) amyloidosis. J Clin Oncol 28:1031-1037, 2010. http ...
An immunoglobulin light chain protein was isolated from the urine of an individual (BRE) with systemic amyloidosis. Complete ... An immunoglobulin light chain protein was isolated from the urine of an individual (BRE) with systemic amyloidosis. Complete ... Tertiary structure of an amyloid immunoglobulin light chain protein: a proposed model for amyloid fibril formation.. Schormann ... From the packing diagram, a model for the amyloid light chain (AL) fibril is proposed based on a pseudohexagonal spiral ...
... a malignant hematologic disorder that can produce significant amounts of monoclonal immunoglobulin free light chains (FLCs). ... occurred in vivo and were produced specifically by the generation of hydrogen peroxide by the VL domain of the light chain. To ... The present study demonstrated that during the process of metabolism of immunoglobulin FLCs, ROS activated the STAT1 pathway in ... that feature increased circulating levels of monoclonal immunoglobulin fragments that require metabolism by the kidney. ...
Serological levels of free immunoglobulin light chains (FLCs), produced in excess of heavy chains during synthesis of ... Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients. Umberto Basile,1 Mariapaola Marino,2 Cecilia ... We found a statistically significant increase in free κ chains in both AChR- and MuSK-MG patients, while free λ chain levels ... We also observed a significant reduction of both free κ and λ chains in 1/4 MuSK-MG patients along with specific abs titer, two ...
Systemic light chain amyloidosis (AL) is caused by the production of toxic light chains and can be fatal, yet effective ... Here the authors show that a machine learning platform analyzing light chain somatic mutations allows the prediction of light ... pathogenic monoclonal immunoglobulin light chains (LC) form toxic aggregates and amyloid fibrils in target organs. Prompt ... chain toxicity to serve as a possible tool for early diagnosis of AL. ...
There is provided an isolated immunoglobulin comprising two heavy polypeptide chains sufficient for the formation of a complete ... light chain in the four-chain model immunoglobulins is that of a committed heavy chain chaperon and that the emergence of light ... As all three immunoglobulins bind to Protein A we refer to them as IgG: namely IgG1 (light chain and heavy chain γ1 (50 Kd) ... The immunoglobulins of the invention, which are devoid of light chains are such that the variable domains of their heavy chains ...
For the quantitative measurement of human IgG heavy chain and light chain intact immunoglobulin in serum. The IgG HLC ratio can ... Immunoglobulin G (IgG) Heavy and Light Chain (HLC) Pairs, κ and λ With Ratio. TEST: 123550 Test number copied ... Immunoglobulin heavy/light chain ratios improve paraprotein detection and monitoring, identify residual disease and correlate ... Heavy and light chain pair quantitation may be useful for:. 1. Distinguishing between broadly migrating monoclonal proteins and ...
... heavy chains and two Ig light chains. There are two types of light chain in humans: kappa (κ) chain, encoded by the ... thus the two light chains of an individual antibody are identical. Each light chain is composed of two tandem immunoglobulin ... The immunoglobulin light chain is the small polypeptide subunit of an antibody (immunoglobulin). A typical antibody is composed ... Other types of light chains can be found in lower vertebrates, such as the Ig-Light-Iota chain of Chondrichthyes and Teleostei ...
Immunoglobulin heavy and light chain genes rearrange independently at early stages of B cell development.. Ehlich A1, Schaal S ... We report the occurrence of immunoglobulin heavy and light chain gene rearrangement at the stage of large B cell precursors. We ... cell receptor complex nor any gene rearrangement in the heavy chain locus is required for the induction of kappa light chain ... Using this system and employing mutant mice in which the membrane exon of the mu chain, the lambda 5 gene, or the JH locus was ...
High-throughput sequencing of the paired human immunoglobulin heavy and light chain repertoire.. DeKosky BJ1, Ippolito GC, ... High-throughput sequencing of the paired human immunoglobulin heavy and light chain repertoire ... High-throughput sequencing of the paired human immunoglobulin heavy and light chain repertoire ... High-throughput sequencing can identify large numbers of heavy- and light-chain variable regions (V(H) and V(L)) in a given B- ...
... Scand J Immunol. 1993 Jun;37(6):623-6. ... of a major fibril subunit protein showed homology with the variable region of a monoclonal lambda immunoglobulin light chain, ...
High sensitivity cardiac troponin T in patients with immunoglobulin light chain amyloidosis ... High sensitivity cardiac troponin T in patients with immunoglobulin light chain amyloidosis ... assay in patients with immunoglobulin light chain amyloidosis (AL) improves risk prediction. ...
Risk factors for venous thromboembolism in immunoglobulin light chain amyloidosis. Katherine M. Bever, Luke I. Masha, Fangui ... Risk factors for venous thromboembolism in immunoglobulin light chain amyloidosis. Katherine M. Bever, Luke I. Masha, Fangui ... Patients with immunoglobulin light chain amyloidosis are at risk for both thrombotic and bleeding complications. While the ... Immunoglobulin light chain amyloidosis (AL) is a rare systemic disorder resulting from organ deposition of amyloid fibrils ...
Immunogold quantitation of immunoglobulin light chains in renal amyloidosis and kappa light chain nephropathy. Download Prime ... Immunohistochemical study of immunoglobulin light chain amyloidosis with antibodies to the immunoglobulin light chain variable ... Immunogold Quantitation of Immunoglobulin Light Chains in Renal Amyloidosis and Kappa Light Chain Nephropathy. Am J Pathol. ... Immunogold quantitation of immunoglobulin light chains in renal amyloidosis and kappa light chain nephropathy. Am J Pathol. ...
Ig Light Chains Generate H2O2 in HK-2 Cells. Overnight incubation of HK-2 cells with each of the light chains (5 mg/ml) ... For light chains, this process has been characterized in some detail. Proximal tubular reabsorption of light chains sparks the ... Because there were limited amounts of some of the light chains, two light chains (κ1 and κ2) were used to demonstrate a dosage- ... For example, light chains that promote light chain deposition disease and amyloidosis undergo endocytosis into mesangial cells ...
Immunoglobulins. Antibodies. Immunoglobulin Light Chains. Anti-Inflammatory Agents. Antiemetics. Autonomic Agents. Peripheral ... Amyloidosis Immunoglobulin Light Chain Deposition Drug: Bortezomib Drug: Dexamethasone Drug: Ibrutinib Other: Laboratory ... Immunoglobulin Light-chain Amyloidosis. Amyloidosis. Proteostasis Deficiencies. Metabolic Diseases. Neoplasms, Plasma Cell. ... how well ibrutinib with or without bortezomib and dexamethasone works in treating patients with immunoglobulin light chain ...
Objectives: Serum immunoglobulin free light chains (FLCs) are frequently elevated in B-cell-mediated autoimmune diseases, ... Serum immunoglobulin free light chains are sensitive biomarkers for monitoring disease activity and treatment response in ...
Highly related immunoglobulin light chain sequences in different multiple sclerosis patients. J Neuroimmunol 1999;100:98-101. ... Highly sensitive, automated immunoassay for immunoglobulin free light chains in serum and urine. Clin Chem 2001;47:673-80. ... Multiple sclerosis: assay of free immunoglobulin light chains. Ann Clin Biochem 2017;54:5-13.doi:10.1177/0004563216652175 ... Objective To determine whether the ratio of cerebrospinal fluid (CSF) immunoglobulin kappa to lambda light chains at time of ...
immunoglobulin; light chain; VL gene; diversity; evolution; Siberian sturgeon; Acipenser baeri; HEAVY-CHAIN; SOMATIC MUTATION; ... Variability of the immunoglobulin light chain in the Siberian sturgeon, Acipenser baeri. Lundqvist, ML Uppsala University. ...
Evidence for one immunoglobulin light-chain type in chickens: absence of a blocked N-terminal light-chain type. Biochem Genet. ... and two identical light chains (IGL). Most tetrapod species encode more than one isotype of light chains. Chicken is the only ... Analysis of the Immunoglobulin Light Chain Genes in Zebra Finch: Evolutionary Implications. Sabyasachi Das,*,1,2 Uzra Mohamedy, ... Organization of the human immunoglobulin lambda light-chain locus on chromosome 22q11.2. Hum Mol Genet. 1995;4:983-991. [PubMed ...
... is a rare and typically fatal disease caused by misfolded light chains-protein chains formed by plasma cells that misfold into ... Immunoglobulin light-chain amyloidosis (AL amyloid-osis), one of the most common forms of amyloidosis, ... Immunoglobulin light-chain amyloidosis (AL amyloid-osis), one of the most common forms of amyloidosis, is a rare and typically ... BACKGROUND: Immunoglobulin light-chain amyloidosis (AL amyloidosis) is a rare and often fatal disease for which there is ...
... light chain in the recognition mechanism of the IG. The diversity of the structural repertoire for immunoglobulin heavy chain ... 1997) Follicular lymphoma immunoglobulin kappa light chains are affected by the antigen selection process, but to a lesser ... Brauninger A, Goossens T, Rajewsky K, Kuppers R. (2001) Regulation of immunoglobulin light chain gene rearrangements during ... Information on clonotypic light-chain expression was available in 35/43 cases: 20/43 expressed kappa chain, whereas 15/43 cases ...
... encoding homogeneous lambda-like light chain. In addition, sterlet possesses sigma-like VL and J-CL genes, which are ... encoding homogeneous lambda-like light chain. In addition, sterlet possesses sigma-like VL and J-CL genes, which are ... The isotype described previously as the teleostean IgL lambda turned out to be a kappa and lambda chain paralog that emerged ... The isotype described previously as the teleostean IgL lambda turned out to be a kappa and lambda chain paralog that emerged ...
Immunoglobulin M (IgG, IgM) (Heavy & Light Chain) antibody (Alkaline Phosphatase (AP)) ABIN637975 from antibodies-online ... anti-Immunoglobulin G, Immunoglobulin M (IgG, IgM) (Heavy & Light Chain) antibody (Alkaline Phosphatase (AP)) from antibodies- ... Itemanti-Immunoglobulin G, Immunoglobulin M (IgG, IgM) (Heavy & Light Chain) antibody (Alkaline Phosphatase (AP)) ... anti-Immunoglobulin G, Immunoglobulin M (IgG, IgM) (Heavy & Light Chain) antibody (Alkaline Phosphatase (AP)) ...
Noncovalent association of heavy and light chains of human immunoglobulins. III. Specific interactions between VH and VL.. C ... Noncovalent association of heavy and light chains of human immunoglobulins. III. Specific interactions between VH and VL. ... Noncovalent association of heavy and light chains of human immunoglobulins. III. Specific interactions between VH and VL. ... Noncovalent association of heavy and light chains of human immunoglobulins. III. Specific interactions between VH and VL. ...
Immunoglobulin light chain (AL) amyloidosis is characterized by tissue deposition of amyloid fibers derived from immunoglobulin ... Immunoglobulin light chain (AL) amyloidosis is characterized by tissue deposition of amyloid fibers derived from immunoglobulin ... Immunoglobulin light chain (AL) amyloidosis is characterized by tissue deposition of amyloid fibers derived from immunoglobulin ... Genome-wide association study of immunoglobulin light chain amyloidosis in three patient cohorts : Comparison with myeloma. Da ...
An internal molecular deletion occurring in a human lambda type immunoglobulin light (L)-chain (Sm lambda) has been defined by ... Primary structure of a deleted human lambda type immunoglobulin light chain containing carbohydrate: protein Sm lambda. ... Primary structure of a deleted human lambda type immunoglobulin light chain containing carbohydrate: protein Sm lambda. ... and light (L) chains and the hypervariable regions, are particularly susceptible to breakage and reunion. We postulate that the ...
... urine free light chain excretion and serum free light chain ratio).. Hematologic responses were measured at three, six, nine ... Immunoglobulin light chain amyloidosis (AL) is a plasma cell dyscrasia characterized by deposition of amyloid fibrils in ... Melphalan, lenalidomide and dexamethasone for the treatment of immunoglobulin light chain amyloidosis: results of a phase II ... Melphalan, lenalidomide and dexamethasone for the treatment of immunoglobulin light chain amyloidosis: results of a phase II ...
Immunoglobulin Free Light Chains Kappa and Lambda. By Technical Data. Immunoglobulin Free Light Chains Kappa and Lambda - ... Immunoglobulin Free Light Chains Kappa and Lambda - for Plasma-EDTA - Immunoassays. Immunoglobulin Free Light Chains Kappa and ... Immunoglobulin Free Light Chains Kappa and Lambda - for Urine - Immunoassays. Immunoglobulin Free Light Chains Kappa and Lambda ... Human - Immunoglobulin Free Light Chains Kappa and Lambda - Immunoassays. Immunoglobulin Free Light Chains Kappa and Lambda - ...
Immunoglobulin lambda light chain orphons on human chromosome 8q11.2. Frippiat, J. P.; Dard, P.; Marsh, S.; Winter, G.; Lefranc ... organization; locus; immunoglobulin; localization; constant region genes; chromosome 8q11.2; heavy-chain; insitu hybridization ... locus has undergone considerably less evolutionary shuffling than either the human light chain kappa locus or the heavy chain ...
... heavy chain, light chain, etc.). Description: Subject matter involving an immunoglobulin or antibody fragment.. Patents under ... Feline immunoglobulin E molecules and related methods. Sep. 1, 2009. 7547436. Receptor specific transepithelial transport of ... Name: Drug, bio-affecting and body treating compositions > Involving immunoglobulin or antibody fragment (e.g., f(ab`)2, fab`, ... Method of obtaining immunoglobulins from colostrum and their use in pharmaceutical composition. Jul. 14, 1998. ...
  • An immunoglobulin light chain protein was isolated from the urine of an individual (BRE) with systemic amyloidosis. (rcsb.org)
  • In systemic light chain amyloidosis (AL), pathogenic monoclonal immunoglobulin light chains (LC) form toxic aggregates and amyloid fibrils in target organs. (nature.com)
  • Systemic light-chain amyloidosis (AL) is a monoclonal gammopathy characterized by the abnormal proliferation of a plasma cell clone producing large amounts of pathogenic immunoglobulin free light chains (LCs) 1 . (nature.com)
  • The identification of an 11-1F4-related mimotope should facilitate development of pan-light chain fibril-reactive antibodies that could be used in the diagnosis and treatment of patients with AL amyloidosis. (nih.gov)
  • Objectives To define whether the high sensitivity cardiac troponin T (hs-cTnT) assay in patients with immunoglobulin light chain amyloidosis (AL) improves risk prediction. (bmj.com)
  • By quantitative immunoelectron microscopy using protein A-gold, the authors compared the content and distribution of immunoglobulin light chain (LC) antigens in glomeruli from 11 cases of renal amyloidosis with that in two cases of kappa LC glomerulopathy and two cases of diabetic glomerulosclerosis. (unboundmedicine.com)
  • TY - JOUR T1 - Immunogold quantitation of immunoglobulin light chains in renal amyloidosis and kappa light chain nephropathy. (unboundmedicine.com)
  • Patients with immunoglobulin light chain amyloidosis are at risk for both thrombotic and bleeding complications. (haematologica.org)
  • This retrospective study examined the frequency of venous thromboembolism in 929 patients with immunoglobulin light chain amyloidosis presenting to a single referral center, correlated risk of venous thromboembolism with clinical and laboratory factors, and examined complications of anticoagulation in this population. (haematologica.org)
  • Immunoglobulin light chain amyloidosis (AL) is a rare systemic disorder resulting from organ deposition of amyloid fibrils comprised of immunoglobulin light chains, typically associated with an underlying clonal plasma cell or lymphoproliferative disorder. (haematologica.org)
  • AL amyloidosis is a rare progressive disease caused by proteotoxic light chain protein produced by small plasma cell clone. (clinicaltrials.gov)
  • This phase II trial studies how well ibrutinib with or without bortezomib and dexamethasone works in treating patients with immunoglobulin light chain amyloidosis that has come back after a period of improvement or that does not respond to treatment. (clinicaltrials.gov)
  • Immunoglobulin light-chain amyloidosis (AL amyloidosis) is a rare and often fatal disease for which there is currently no treatment approved by the US Food and Drug Administration or the European Medicines Agency. (ahdbonline.com)
  • Immunoglobulin light-chain amyloidosis (AL amyloid-osis), one of the most common forms of amyloidosis, is a rare and typically fatal disease caused by misfolded light chains-protein chains formed by plasma cells that misfold into an abnormal shape-that form soluble toxic aggregates and deposit fibrils (amyloid) in organs. (ahdbonline.com)
  • Immunoglobulin light chain (AL) amyloidosis is characterized by tissue deposition of amyloid fibers derived from immunoglobulin light chain. (lu.se)
  • These data provide evidence for common genetic susceptibility to AL amyloidosis and MM. Cyclin D1 is a more prominent driver in AL amyloidosis than in MM, but the links to aggregation of light chains need to be demonstrated. (lu.se)
  • Accurate measurement of monoclonal free light chains in serum and/or urine is especially important in light-chain diseases, such as light-chain myeloma, primary systemic amyloidosis, and light chain-deposition disease. (biovendor.com)
  • Immunoglobulin light chain amyloidosis (AL) is a plasma cell dyscrasia characterized by deposition of amyloid fibrils in various organs and tissues, derived from monoclonal light chains, leading to organ dysfunction. (haematologica.org)
  • Immunoglobulin light chain amyloidosis (AL) is characterized by a limited clonal expansion of plasma cells and amyloid formation. (springer.com)
  • AL-Base, a curated database of human immunoglobulin (Ig) light chain (LC) sequences derived from patients with AL amyloidosis and controls, is described, along with a collection of analytical and graphic tools designed to facilitate their analysis. (semanticscholar.org)
  • Clarifying immunoglobulin gene usage in systemic and localized immunoglobulin light-chain amyloidosis by mass spectrometry. (semanticscholar.org)
  • Structure and energetic basis of overrepresented λ light chain in systemic light chain amyloidosis patients. (semanticscholar.org)
  • Inhibition of Light Chain 6aJL2-R24G Amyloid Fiber Formation Associated with Light Chain Amyloidosis. (semanticscholar.org)
  • Proteomic detection of immunoglobulin light chain variable region peptides from amyloidosis patient biopsies. (semanticscholar.org)
  • Immunoglobulin D lambda multiple myeloma and amyloidosis with predominant soft tissue involvement. (semanticscholar.org)
  • Depending on the spatial arrangement of the secreted proteins, light chain-derived amyloidosis (AL) can be distinguished from non-amyloid light chain deposition disease (LCDD). (biomedcentral.com)
  • Systemic immunoglobulin light chain amyloidosis is a protein misfolding disease caused by the conversion of immunoglobulin light chains from their soluble functional states into highly organized amyloid fibrillar aggregates that lead to organ dysfunction. (ucl.ac.uk)
  • Aids in the diagnosis and monitoring of multiple myeloma, lymphocytic neoplasms, Waldenstrom's macroglobulinemia, amyloidosis, light chain deposition disease and connective tissue diseases such as systemic lupus erythematosus. (umich.edu)
  • multiple myeloma), primary amyloidosis and light chain deposition disease. (umich.edu)
  • Immunoglobulin light-chain (AL) amyloidosis is a systemic amyloidosis characterized by extracellular accumulation of fibrillary deposits composed of monoclonal immunoglobulin light chain fragments. (paom.pl)
  • The aim of the study is to present the possibilities of using a standard electrocardiography and an echocardiography for diagnosis of a rare immunoglobulin AL amyloidosis. (paom.pl)
  • The primary λ-light chain amyloidosis of heart, kidneys, autonomic nerves and soft tissue was diagnosed. (paom.pl)
  • We presented a typical case of immunoglobulin AL amyloidosis, which was detected thanks to characteristic electro- and echocardiographic findings. (paom.pl)
  • Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18-22 April 2004. (paom.pl)
  • This amyloid light-chain (AL) amyloidosis, which is a hereditary type of amyloidosis and not the result of a monoclonal plasma cell dyscrasia, may be misdiagnosed and lead to inappropriate chemotherapy. (elsevier.com)
  • Researchers developed a risk stratification model for prognosis in systemic immunoglobulin light-chain (AL) amyloidosis with renal involvement, with results published in the British Journal of Haematology . (hematologyadvisor.com)
  • A risk stratification for systemic immunoglobulin light-chain amyloidosis with renal involvement [published online July 26, 2019]. (hematologyadvisor.com)
  • Different light chains are responsible for two diseases with diametrically opposite mesangial alterations: Light chain deposition disease which results in the expansion of the mesangium due to accumulation of matrix proteins not present in the normal mesangium and AL (light chain-associated) amyloidosis where the native mesangial matrix is replaced by fibrils (amyloid). (heraldopenaccess.us)
  • The glomerulopathies associated with monoclonal light chains are Light Chain Deposition Disease (LCDD) and AL (light chainrelated) amyloidosis, each creating completely different alterations in the mesangium. (heraldopenaccess.us)
  • Amyloid light chain (AL) amyloidosis is the most common of these diseases, and is caused by the clonal expansion of a plasma cell that overproduces an immunoglobulin light chain, which forms fibrils upon secretion into the extracellular space. (biologists.com)
  • One of the key questions in the field of AL amyloidosis is how the light chain sequence correlates with its aggregation propensity. (biologists.com)
  • Gertz MA, Buadi FK, Lacy MQ, Hayman SR. Immunoglobulin light-chain amyloidosis (primary amyloidosis). (medlineplus.gov)
  • Background: Immunoglobulin free light chains (FLC) form the substrate for synthesis of amyloid fibrils in patients with AL amyloidosis. (ashpublications.org)
  • [email protected]#To evaluate the clinical characteristics and outcomes of very high risk patients with primary immunoglobulin light-chain amyloidosis (pAL) at a single center in China . (bvsalud.org)
  • Predicting the onset of AL is highly challenging, as each patient carries a different pathogenic LC sequence resulting from a unique rearrangement of variable (V) and joining (J) immunoglobulin genes and a unique set of somatic mutations (SMs) acquired during B cell affinity maturation 9 (Fig. 1a ). (nature.com)
  • The immunoglobulin light chain genes in tetrapods can be classified into three distinct groups: kappa (κ), lambda (λ), and sigma (σ). (wikipedia.org)
  • Specific rearrangement of lambda light chain of immunoglobulins can lead to loss of some protein coding genes, which does not seem to be functionally relevant (while functionally relevant miR-650 can be overexpressed). (wikipedia.org)
  • Immunoglobulin heavy and light chain genes rearrange independently at early stages of B cell development. (nih.gov)
  • Human SMZ B cells are a heterogeneous population: evidence for this heterogeneity was provided by mutation analysis of rearranged immunoglobulin heavy (IGH) chain genes of microdissected SMZ cells as well as tonsillar marginal zone B cells (equivalent to SMZ B cells) ( 4 , 5 , 6 ), which has demonstrated that some cells carried mutated IGHV genes while other cells carried unmutated genes. (springer.com)
  • The aim of this study was to fill important gaps in the evolutionary history of immunoglobulins by examining the structure and diversity of IgL genes in non-teleost ray-finned fish. (frontiersin.org)
  • These structural studies and other findings suggest that restricted areas in the DNA of immunoglobulin genes, such as the hinge regions of heavy (H) and light (L) chains and the hypervariable regions, are particularly susceptible to breakage and reunion. (eurekamag.com)
  • These studies provide additional support for the hypothesis and two distinct structural genes encode a single immunoglobulin polypeptide chain. (eurekamag.com)
  • As there is no other evidence for Vh genes outside the major locus, it appears that the human lambda locus has undergone considerably less evolutionary shuffling than either the human light chain kappa locus or the heavy chain locus. (cnrs.fr)
  • The unique combination of heavy and light chain genes defines the immunological activity of a B cell and also its identity, also referred to as its clonotype. (biomedcentral.com)
  • While the achievable depth of these amplicon-based approaches provided remarkable resolution (10 5 -10 6 chains in a single experiment) [ 8 ], a significant limitation of this technology for functional studies of the immune system is that it only sequences a single chain and cannot provide information on endogenous pairing of IgH/IgL genes to definitively identify a B cell clonotype. (biomedcentral.com)
  • Single-cell PCR and sequencing of full-length Ig heavy (Igh) and Igk and Igl light chain genes is a powerful tool to measure the diversity of antibody repertoires and allows the functional assessment of B-cell responses through direct Ig gene cloning and the generation of recombinant mAbs. (mpg.de)
  • The murine immunoglobulin kappa (κ) light-chain multigene family includes the constant region (Cκ), joining-region genes, and approximately 30 kappa-variable (Vκ) region families. (elsevier.com)
  • Hatton, KS & Schildkraut, CL 1990, ' The mouse immunoglobulin kappa light-chain genes are located in early- and late-replicating regions of chromosome 6 ', Molecular and cellular biology , vol. 10, no. 8, pp. 4314-4323. (elsevier.com)
  • The immunoglobulin light chain is the small polypeptide subunit of an antibody (immunoglobulin). (wikipedia.org)
  • A typical antibody is composed of two immunoglobulin (Ig) heavy chains and two Ig light chains. (wikipedia.org)
  • Sharks also possess, as part of their adaptive immune systems, a functional heavy-chain homodimeric antibody-like molecule referred to as IgNAR (immunoglobulin new antigen receptor). (wikipedia.org)
  • Only one type of light chain is present in a typical antibody, thus the two light chains of an individual antibody are identical. (wikipedia.org)
  • A typical immunoglobulin (antibody, Ig) in jawed vertebrates is composed of two identical heavy chains (IGH) and two identical light chains (IGL) and provides defense against all extracellular and some intracellular pathogens ( Klein and Hořejší 1997 ). (pubmedcentralcanada.ca)
  • Subject matter involving an immunoglobulin or antibody fragment. (patentgenius.com)
  • Analysis of the structural correlates for antibody polyreactivity by multiple reassortments of chimeric human immunoglobulin heavy and light chain V segments. (rupress.org)
  • We analyzed the contribution of Ig polyvalency and of heavy (H) and light (L) chain variable (V) regions to polyreactivity in recombinatorial experiments involving the VH-diversity(D)-JH and V kappa-J kappa gene segments of a human polyreactive IgM, monoclonal antibody 55 (mAb55), and those of a human monoreactive anti-insulin IgG, mAb13, in an in vitro C gamma l and C kappa human expression system. (rupress.org)
  • According to the four-chain model, the antigen binding site of an antibody is located in the variable domains of each of the heavy and light chains, and requires the association of the heavy and the light chains variable domains. (justia.com)
  • The kappa light chain antibody labels kappa light chain expressing B lymphocytes and plasma cells. (quartett.com)
  • The pairing of an anti-lambda with a kappa light chain antibody is useful for identifying monoclonality of lymphoid malignancies. (quartett.com)
  • The lambda light chain antibody labels the lambda light chain that expresses normal and neoplastic B lymphocytes as well as plasma cells. (quartett.com)
  • This antibody reacts to the kappa light chain of mouse immunoglobulins. (revmab.com)
  • Here we developed a two-dimensional bar-coded primer matrix to combine Igh and Igk/Igl chain gene single-cell PCR with next-generation sequencing for the parallel analysis of the antibody repertoire of over 46 000 individual B cells. (mpg.de)
  • Several different types of heavy chain exist that define the class or isotype of an antibody. (wikipedia.org)
  • This heavy chain, identified in both rainbow trout (τ) and zebrafish (ζ), could potentially form a distinct antibody isotype (IgT or IgZ) that may precede IgM in evolutionary terms. (wikipedia.org)
  • The latter type is a heavy-chain antibody, an antibody lacking light chains, and can be used to produce single-domain antibodies, which are essentially the variable domain (VNAR) of an IgNAR. (wikipedia.org)
  • Only the nonhybridized RNA gave a T1 ribonuclease fingerprint showing oligonucleotides derived from the variable and constant regions of the light chain messenger RNA. (ox.ac.uk)
  • In addition, this fingerprint showed oligonucleotides derived from the untranslated regions of the light chain messenger RNA. (ox.ac.uk)
  • abstract = "The immunoglobulin λ isotype is present in nearly all vertebrates and plays an important role in the human immune system. (edu.sa)
  • For the quantitative measurement of human IgG heavy chain and light chain intact immunoglobulin in serum. (labcorp.com)
  • 1. Distinguishing between broadly migrating monoclonal proteins and restricted polyclonal immunoglobulin patterns on serum protein electrophoresis. (labcorp.com)
  • Heavy and light chain (HLC) quantitation should be used as a complementary method to serum protein electrophoresis. (labcorp.com)
  • International guidelines recommend serum protein electrophoresis or nephelometric immunoglobulin quantification as tools to monitor patients' disease 1 (alongside other tests including flow cytometry and serum free light chain analysis). (labcorp.com)
  • In a healthy individual, the total kappa-to-lambda ratio is roughly 2:1 in serum (measuring intact whole antibodies) or 1:1.5 if measuring free light chains, with a highly divergent ratio indicative of neoplasm. (wikipedia.org)
  • In healthy individuals, the majority of light chains in serum exist bound to heavy chain. (biovendor.com)
  • However, low levels of free light chains (FLCs) are found in serum of normal individuals due to their excess production over heavy chains by mature B-cells. (biovendor.com)
  • It is the most abundant immunoglobulin and is approximately equally distributed in blood and in tissue liquids, constituting 75% of serum immunoglobulins in humans. (vitalcoaching.com)
  • Elevated serum levels of monoclonal free light chains are associated with malignant plasma cell proliferation (eg. (umich.edu)
  • Raised serum levels of polyclonal free light chains may be associated with automimmune diseases such as systemic lupus erythematosus. (umich.edu)
  • Isolation and characterization of κ light-chain protein from serum of an affected member of the kindred revealed mutation in the constant region of kκlight-chain, with cysteine replacing serine at amino acid residue 131. (elsevier.com)
  • A decade or so ago, the first automated assay was launched for the quantitation of serum κ and λ immunoglobulin free light chains (FLC). (bath.ac.uk)
  • In the present study, we investigated the serum levels of kappa and lambda free light chains (FLC) of immunoglobulins and the kappa/lambda FLC ratio in Brazilian patients with chronic HCV infection and cryoglobulinemia . (bvsalud.org)
  • The relationship of the light chain types and their levels in serum to the clinical feature at presentation and the eventual outcome has not been systematically studied. (ashpublications.org)
  • Median values of serum cTnI, NT-proBNP, and free light chains difference were 0.25 μg/L, 11 733 ng/L, and 403 mg/L, respectively. (bvsalud.org)
  • Because of the less-than-robust response to therapy and impact on choice of optimal chemotherapy and prognosis, chronic kidney disease has drawn attention in the treatment of multiple myeloma, a malignant hematologic disorder that can produce significant amounts of monoclonal immunoglobulin free light chains (FLCs). (jci.org)
  • To the extent that the experiments reflect the human condition, these studies offer insights into the pathogenesis of progressive kidney failure in the setting of lymphoproliferative disorders, such as multiple myeloma, that feature increased circulating levels of monoclonal immunoglobulin fragments that require metabolism by the kidney. (jci.org)
  • Ig light chains produced in neoplastic plasma cells, such as in multiple myeloma, are called Bence Jones proteins. (wikipedia.org)
  • H. Ludwig, D. Milosavljevic, N. Zojer, J.M. Faint, A.R. Bradwell, W. Hübl, S.J. Harding Immunoglobulin heavy/light chain ratios improve paraprotein detection and monitoring, identify residual disease and correlate with survival in multiple myeloma patients . (eurofins-biomnis.com)
  • There are two types of light chain in humans: kappa (κ) chain, encoded by the immunoglobulin kappa locus ([email protected]) on chromosome 2 lambda (λ) chain, encoded by the immunoglobulin lambda locus ([email protected]) on chromosome 22 Antibodies are produced by B lymphocytes, each expressing only one class of light chain. (wikipedia.org)
  • Camelids are unique among mammals as they also have fully functional antibodies which have two heavy chains, but lack the light chains usually paired with each heavy chain. (wikipedia.org)
  • IgNAR is believed to have never had an associated light chain, contrary to the understanding that the heavy-chain-only antibodies in camelids may have lost its light chain partner through evolution. (wikipedia.org)
  • In vivo analysis of these tumors with monoclonal antibodies detected the expression of the B-cell surface markers immunoglobulin M (IgM), CIa, Bu2, and CLA-1, but not IgG, Bu1, or a T-cell surface marker, CT-1. (asm.org)
  • Among the proteins forming the class of the immunoglobulins, most of them are antibodies and accordingly present an antigen binding site or several antigen binding sites. (justia.com)
  • The Hevylite reagents, which are made up of polyclonal antibodies, recognise the epitopes which are only found at the whole immunoglobulin heavy and light chain junction. (eurofins-biomnis.com)
  • These antibodies allow us to quantify the immunoglobulin pairs (IgG kappa/IgG lambda, IgA kappa/IgA lambda, IgM kappa/IgM lambda) by using nephelometry or turbidimetry and a ratio calculation between the monoclonal component and the residual immunoglobulins of the same class. (eurofins-biomnis.com)
  • Antibodies, also referred to as immunoglobulins (Ig) or B cell antigen receptors (BCRs), are produced by the paired expression of a "heavy chain" (IgH) immunoglobulin gene and a "light chain" (IgL) immunoglobulin gene. (biomedcentral.com)
  • NanoTag's FluoTag®-X2 species-specific anti-immunoglobulin secondary tools are alpaca single-domain antibodies, also referred to as Nanobodies® (trademark of Ablynx, Inc). With only ~15kD, they are 10-times smaller than conventional IgGs. (nano-tag.com)
  • Bence-Jones proteins are a part of regular antibodies called light chains. (medlineplus.gov)
  • Sometimes, when your body makes too many antibodies, the level of light chains also rises. (medlineplus.gov)
  • Immunoglobulin light chains have two similar domains, each with a hydrophobic primary surrounded by antibodies (Sigma, St. Lakewood, NJ) (Chung et al. (stopvivisection.info)
  • Several methods have been developed to analyze Immunoglobulin (Ig) chains of single B or plasma cells 1-4 , but it is usually not known which particular cell secretes disease-relevant expanded antibodies and which cell produces irrelevant Ig chains. (researchsquare.com)
  • Cows, specifically Bos taurus, show a variation on the general mammalian theme in which the heavy chain CDR H3 region has adapted to produce a divergent repertoire of antibodies which present a "stalk and knob" antigen interaction surface instead of the more familiar bivalent tip surface. (wikipedia.org)
  • The resulting antibodies are designated IgW (also called IgX or IgNARC) and IgNAR (immunoglobulin new antigen receptor). (wikipedia.org)
  • Taken together, these results have provided evidence for the structural basis of the specificity of 11-1F4 for both kappa and lambda light chain fibrils. (nih.gov)
  • This plasma cell dyscrasia is characterized by monoclonal plasma cell's excessive production of monoclonal immunoglobulin light-chains that tends to misfold and subsequently deposit as amyloid fibrils in visceral organs. (clinicaltrials.gov)
  • Light chain fibrils were isolated from heart tissue of an AL patient, and the cryo-EM structure was solved at 4.0 Åresolution. (biologists.com)
  • First however, the composition of the fibrils was assessed, and the authors identified a mixture of different lengths of the light chain, ranging from the full-length protein to just the variable domain, V L . Limited proteolysis showed that the V L domain was protected against degradation, suggesting that it formed the fibril core, whereas the C-terminal parts were efficiently degraded by the protease. (biologists.com)
  • A previous study on patient-derived light chain fibrils at lower resolution showed that the fibrils were polymorphic 3 , i.e. existing in distinct structural forms, but in this case only one type of structure was identified on the EM grids. (biologists.com)
  • a) Light chain fibrils extracted from the AL patient's heart tissue on the EM grid. (biologists.com)
  • High-throughput sequencing of the paired human immunoglobulin heavy and light chain repertoire. (nih.gov)
  • Human immunoglobulin molecules consist of two identical heavy chains which define immunoglobulin classes (IgG, IgA, IgM, IgD and IgE) and identical light chains (kappa or lambda) that are covalently linked to a heavy chain. (biovendor.com)
  • The exact normal ratio of kappa to lambda, according to a novel polyclonal free light chain assay, ranges from 0.26 to 1.65. (wikipedia.org)
  • It is important to note that, in contrast to increased levels in lymphoma patients, these Ig light chains are polyclonal. (wikipedia.org)
  • The approximate 500 mg of free light chains that is produced by the normal lymphoid system daily is filtered and catabolized by the kidney in the proximal tubule, such that only approximately 1 to 10 mg of polyclonal light chains normally appears in the urine ( 4 - 8 ). (asnjournals.org)
  • Dispenzieri, A 2012, ' Polyclonal immunoglobulin free light chain and chronic inflammation ', Mayo Clinic Proceedings , vol. 87, no. 10, pp. 1033. (elsevier.com)
  • There is provided an isolated immunoglobulin comprising two heavy polypeptide chains sufficient for the formation of a complete antigen binding site or several antigen binding sites, wherein the immunoglobulin is further devoid of light polypeptide chains. (freepatentsonline.com)
  • The Sm lambda polypeptide chain has an approximate molecular weight of 15,000 and contains 135 amino-acid residues. (eurekamag.com)
  • The invention relates to new isolated immunoglobulins which are devoid of light polypeptide chains. (justia.com)
  • These immunoglobulins do not consist in the degradation products of immunoglobulins composed of both heavy polypeptide and light polypeptide chains but to the contrary, the invention defines a new member of the family of the immunoglobulins, especially a new type of molecules capable of being involved in the immune recognition. (justia.com)
  • Up to now the structure proposed for immunoglobulins consists of a four-chain model referring to the presence of two identical light polypeptide chains (light chains) and two identical heavy polypeptide chains (heavy chains) linked together by disulfide bonds to form a y- or T-shaped macromolecules. (justia.com)
  • The two heavy polypeptide chains are usually linked by disulphide bounds in a so-called "hinge region" situated between the first and second domains of the constant region. (justia.com)
  • E. S. Ward et al (1) have described some experiments performed on variable domains of heavy polypeptide chains (V.sub.H) or/and light polypeptide chains (V.sub.K /F.sub.V) to test the ability of these variable domains, to bind specific antigens. (justia.com)
  • Although saturation of the multiligand endocytotic receptor complex of the proximal tubule permits light chains to appear in the tubule fluid of the distal nephron and finally in the urine, overproduction of monoclonal light chains can result in significant concentration of these proteins in the proximal tubule. (asnjournals.org)
  • The noncovalent interaction between the VH fragments and autologous kappa-chains was studied by ultraviolet difference spectroscopy and circular dichroism. (jimmunol.org)
  • In contrast to the studies with autologous species, the VH fragments did not associate with heterologous kappa-chains as judged both by difference spectroscopy and gel filtration using radiolabeled VH fragments. (jimmunol.org)
  • Reference is especially made to the part concerning the definition of the four-chain immunoglobulins, their polypeptidic and genetic structures, the definition of their variable and constant regions and the obtention of the fragments produced by enzymatic degradation according to well known techniques. (justia.com)
  • The invention does not start from the idea that the different fragments (light and heavy chains) and the different domains of these fragments of four-chain model immunoglobulin can be modified to define new or improved antigen binding sites or a four-chain model immunoglobulin. (justia.com)
  • As a result, bacterial clones were selected which contained gene fragments showing the ability to hybridize with the RNA fraction characterized by mRNA mobility of the light-chain immunoglobulin G. (elsevier.com)
  • Each light chain is composed of two tandem immunoglobulin domains: one constant (CL) domain one variable domain (VL) that is important for binding antigen The approximate length of a light chain protein is from 211 to 217 amino acids. (wikipedia.org)
  • In this regard, we previously had reported that mAb 11-1F4, generated by immunizing mice with a thermally denatured variable domain (VL) fragment of the human kappa4 Bence Jones protein Len, bound to a non-native conformational epitope located within the N-terminal 18 residues of fibrillar, as well as partially denatured, Ig light chains (O'Nuallain, B., et al. (nih.gov)
  • Comparison of Vkappa and Vlambda N-terminal germline consensus sequences with protein Len and 11-1F4-binding phages indicated that this antibody's cross-reactivity with light chains was related to an invariant proline at position(s) 7 and/or 8, bulky hydrophobic residues at positions 11 and 13, and additionally, to the ability to accommodate amino acid diversity at positions 1-4. (nih.gov)
  • Amino acid sequence analysis of a major fibril subunit protein showed homology with the variable region of a monoclonal lambda immunoglobulin light chain, subgroup III or IV. (nih.gov)
  • the subsequent addition of lysosomal enzymes permits hydrolysis of the light chain protein and return of the amino acid components to the circulation ( 2 , 3 , 13 ). (asnjournals.org)
  • Intrigued by these observations, this laboratory performed a series of experiments that showed that light chains per se are capable of oxidizing water to form H 2 O 2 and further sufficiently alter cellular redox signaling to stimulate the production of monocyte chemoattractant protein-1 (MCP-1) in cells in culture. (asnjournals.org)
  • Unfolded protein response activation reduces secretion and extracellular aggregation of amyloidogenic immunoglobulin light chain. (semanticscholar.org)
  • Amino acid sequencing of isolated amyloid protein identified sequences compatible with the constant region of the immunoglobulin κ light-chain. (elsevier.com)
  • I did Bence jones protein test and it writed Immunoglobulin Free Light Chains Kappa and Lambda is this bad? (medhelp.org)
  • Pre-B lymphocytes can synthesize heavy chain in the absence of light chain, which then can allow the heavy chain to bind to a heavy-chain binding protein. (wikipedia.org)
  • In all these, light chains purified from the urine of patients with renal biopsy-proven conditions have been used. (heraldopenaccess.us)
  • Using this system and employing mutant mice in which the membrane exon of the mu chain, the lambda 5 gene, or the JH locus was inactivated by gene targeting, we found that expression of the pre-B cell receptor complex is necessary for the transition from the large CD43+ to the small CD43- pre-B cell stage. (nih.gov)
  • We show that neither the pre-B cell receptor complex nor any gene rearrangement in the heavy chain locus is required for the induction of kappa light chain gene rearrangement in early B cell progenitors. (nih.gov)
  • Analysis of the light-chain locus demonstrated that 20 of 22 lines contained a single rearranged allele. (asm.org)
  • We propose that v-rel expression arrests diversification of the light-chain locus in these lymphoid cells, allowing the production of stable, clonal B-cell populations. (asm.org)
  • In non-Hodgkin's lymphoma (NHL), the majority of translocations involve the immunoglobulin heavy chain gene (IGH) locus, while a few involve the immunoglobulin light chain gene (IGL) locus, consisting of the kappa light chain gene (IGkappa) and the lambda light chain gene (IGlambda). (mysciencework.com)
  • ELISA of mouse immunoglobulins shows RM110 reacts to the lambda light chain of mouse immuno- globulins. (revmab.com)
  • During phylogeny, multiple isotypes of both IgH and IgL chains have evolved ( 1 - 4 ). (frontiersin.org)
  • Similar to the situation observed for bony fish, three distinct Ig heavy chain isotypes have been identified in cartilaginous fish. (wikipedia.org)
  • With the exception of μ, these Ig heavy chain isotypes appear to be unique to cartilaginous fish. (wikipedia.org)
  • Third, we shuffled the framework 1 (FR1)-FR3 and complementarity determining region 3 (CDR3) regions of the H and kappa chain V segments of the mAB55-derived IgG molecule with the corresponding regions of the monoreactive IgG mAb13. (rupress.org)
  • The mAb55-derived IgG molecule lost polyreactivity when the H chain CDR3, but not the FR1-FR3 region, was replaced by the corresponding region of mAb13, suggesting that within the H chain, the CDR3 provides the major structural correlate for multiple Ag-binding. (rupress.org)
  • This was formally proved by the multiple Ag-binding of the originally monoreactive mAb13-derived IgG molecule grafted with the mAb55-derived H chain CDR3. (rupress.org)
  • Each immunoglobulin molecule consists of two identical heavy chains and two identical light chains. (quartett.com)
  • Major functions of the proximal tubule include reclamation of filtered low molecular weight proteins, such as Ig light chains, and other essential molecules that are carried by proteins, such as folate ( 1 - 3 ). (asnjournals.org)
  • In these experiments, molecules that contained both heavy and light chains were used but not light chains alone. (asnjournals.org)
  • The Ig molecules are generally composed of heavy (IgH) and light (IgL) chains. (frontiersin.org)
  • The inventors have surprisingly established that different molecules can be isolated from animals which naturally produce them, which molecules have functional properties of immunoglobulins these functions being in some cases related to structural elements which are distinct from those involved in the function of four-chain immunoglobulins due for instance to the absence of light chains. (justia.com)
  • Plasma cells do not produce complete immunoglobulin molecules. (5minuteconsult.com)
  • As low molecular weight proteins, restriction from glomerular filtration is minimized, permitting significant amounts of Ig light chains to be endocytosed into the proximal tubule epithelium, particularly in plasma cell dyscrasias. (asnjournals.org)
  • An internal molecular deletion occurring in a human lambda type immunoglobulin light (L)-chain (Sm lambda) has been defined by sequence analysis. (eurekamag.com)
  • Normal light chains are small molecular weight proteins that are filtered through the peripheral capillary walls and are delivered to the proximal tubules where they are catabolized by endosomal activity in the apical portions of the tubules and their amino acids are then returned to the circulation. (heraldopenaccess.us)
  • From the packing diagram, a model for the amyloid light chain (AL) fibril is proposed based on a pseudohexagonal spiral structure with a rise of approximately the width of two dimers per 360 degree turn. (rcsb.org)
  • Several new classes of drugs, such as proteasome inhibitors and immunomodulatory drugs, along with high-dose chemotherapy and autologous haematopoietic stem cell transplantation, have led to rapid and deep suppression of amyloid light chain production in the majority of patients. (ucl.ac.uk)
  • The variable domain of any heavy chain is composed of a single immunoglobulin domain. (wikipedia.org)
  • Hello, I invite you to consult the site www.biomnis.com, on page Quantification of immunoglobulin heavy and light chain pairs https://www.eurofins-biomnis.com/en/biomnis-live/light-on/quantification-of-immunoglobulin-heavy-and-light-chain-pairs/ Best regards. (eurofins-biomnis.com)
  • The development of FLC specific mAbs is difficult because the mAbs must demonstrate specificity for epitopes that are exposed on FLC but hidden on LC bound to whole immunoglobulin. (bath.ac.uk)
  • 2 Total IgG nephelometric assays will include nontumor immunoglobulin, and measurement of either IgG κ or IgG λ may give a more accurate representation of tumor production. (labcorp.com)
  • Immunoglobulin (IG) gene usage and somatic mutation patterns were studied in a series of 43 SMZL cases. (springer.com)
  • Previous analyses of a cDNA library generated from synovium of RA patient BC revealed immunoglobulin κ light chain transcripts with extensive somatic mutation, frequent N region addition, and unexpected variation in the lengths of CDR3 regions which form the center of the antigen binding site. (uab.edu)
  • This study demonstrated that light chains belonged to a class of proteins that are capable of catalyzing the formation of hydrogen peroxide. (asnjournals.org)
  • Ig light chains, once termed Bence Jones proteins ( 4 ), appear in the circulation as monomers (M r approximately 22 kD) and dimers (M r approximately 44 kD). (asnjournals.org)
  • The detection of urinary monoclonal kappa or lambda free light chains of immunoglobulin, also know as Bence Jones proteins (BJP), are important for identifying and monitoring Bcell malignancies too. (biovendor.com)
  • Even though recognition of certain antigenic epitopes may be critically dependent on conformations achieved by the interaction of both the IG heavy chain (HC) and light chain (LC) proteins, the focus to date has been on the IG HC gene, with studies on CLL IG LCs lagging behind. (linkos.cz)
  • MIDD is characterized by the deposition of light and heavy chain proteins. (biomedcentral.com)
  • Monoclonal immunoglobulin deposition disease (MIDD) is characterized by the deposition of monotypic light and/or heavy chain proteins in various tissues and organs. (biomedcentral.com)
  • Such immunoglobulins can be used for several purposes, especially for diagnosis or therapeutical purposes including protection against pathological agents or regulation of the expression or activity of proteins. (justia.com)
  • Such pH-dependence shows that the proteins conformation is considerably suffering from the titration of acidic and simple aspect chains at severe pH. (stopvivisection.info)
  • Individual B-cells in lymphoid tissue possess either kappa or lambda light chains, but never both together. (wikipedia.org)
  • Using immunohistochemistry, it is possible to determine the relative abundance of B-cells expressing kappa and lambda light chains. (wikipedia.org)
  • Objective To determine whether the ratio of cerebrospinal fluid (CSF) immunoglobulin kappa to lambda light chains at time of multiple sclerosis (MS) diagnosis predicts disease progression and whether this was intrinsic to CSF plasmablasts. (bmj.com)
  • Individual B cells express either kappa or lambda light chains. (quartett.com)
  • Structural elements of immunoglobulin light chains are depicted on top of the sequences (FR1 = framework 1, CDR1 = complementary determining region 1, FR2 = framework 2, CDR2 = complementary determining region 2, FR3 = framework 3, CDR3 = complementary determining region 3, FR4 = framework 4). (nature.com)
  • AL-Base: a visual platform analysis tool for the study of amyloidogenic immunoglobulin light chain sequences. (semanticscholar.org)
  • The complete sequences of one VκI, one VκIII and N-terminal sequences of two VκI light chains, from patients with different forms of Fanconi's syndrome, were compared with four previously studied sequences. (elsevier.com)
  • Here we describe a bioinformatic pipeline, BALDR ( B CR A ssignment of L ineage using D e novo R econstruction) that accurately reconstructs the paired heavy and light chain immunoglobulin gene sequences from Illumina single-cell RNA-seq data. (biomedcentral.com)
  • This copy was completed with another chain without adding the exogenous primer, with the aid of the Klenov fragment of DNA polymerase I. After treatment of the double-stranded pin DNA with endonuclease S1, the poly (dA) sequences were built up using terminal deoxynucleotidyl transferase. (elsevier.com)
  • While only two patients shared a stereotyped heavy-chain complementarity determining region 3 (CDR3) sequence, two kappa chain CDR3 stereotyped sequences accounted for 77% of BCRs. (nature.com)
  • Since this rarely yields full sequence coverage, we reveal the complete sequences by alignment of the peptides to the immunoglobulin transcriptome of the same patient. (researchsquare.com)
  • Other types of light chains can be found in lower vertebrates, such as the Ig-Light-Iota chain of Chondrichthyes and Teleostei. (wikipedia.org)
  • All jawed vertebrates produce immunoglobulins (IGs) as a defense mechanism against pathogens. (pubmedcentralcanada.ca)
  • Immunoglobulins (Ig) are heteromeric glycoproteins that play a crucial role in the humoral immune defense of all jawed vertebrates. (frontiersin.org)
  • The present study demonstrated that during the process of metabolism of immunoglobulin FLCs, ROS activated the STAT1 pathway in proximal tubule epithelium. (jci.org)
  • Serological levels of free immunoglobulin light chains (FLCs), produced in excess of heavy chains during synthesis of immunoglobulins by plasma cells, can be considered a direct marker of B cell activity in different systemic inflammatory-autoimmune conditions and may represent a useful predictor of rituximab (RTX) therapeutic efficacy, as reported for rheumatoid arthritis and systemic lupus erythematosus. (hindawi.com)
  • Each B-cell receptor consists of a pair of heavy and light chains. (nih.gov)
  • Light chains in the tubular fluid are absorbed into the proximal tubule epithelium by binding initially to a receptor that consists of megalin and cubilin ( 9 - 12 ). (asnjournals.org)
  • Sequence analysis of the immunoglobulin antigen receptor of hepatitis C virus-associated non-Hodgkin lymphomas suggests that the malignant cells are derived from the rheumatoid factor-producing cells that occur mainly in type II cryoglobulinemia. (nature.com)
  • Instead, they produce the heavy and light chain components separately and then assemble them before secretion into the bloods. (5minuteconsult.com)
  • Immunoglobulin secretion by plasma cells infiltrating synovial membranes is a prominent feature of RA. (uab.edu)
  • An elevated IgG heavy and light chain (HLC) pair ratio suggests a clonal proliferation of an IgG κ clone of plasma cells. (labcorp.com)
  • We report the occurrence of immunoglobulin heavy and light chain gene rearrangement at the stage of large B cell precursors. (nih.gov)
  • High-throughput sequencing can identify large numbers of heavy- and light-chain variable regions (V(H) and V(L)) in a given B-cell repertoire, but information about endogenous pairing of heavy and light chains is lost after bulk lysis of B-cell populations. (nih.gov)
  • Expression of v-rel induces mature B-cell lines that reflect the diversity of avian immunoglobulin heavy- and light-chain rearrangements. (asm.org)
  • Northern (RNA blot) analysis confirmed abundant expression of v-rel mRNA, and Southern analysis revealed rearrangement of both heavy- and light-chain immunoglobulin loci. (asm.org)
  • Characterization of immunoglobulin heavy and light chain gene expression in chronic lymphocytic leukemia and related disorders. (cdc.gov)
  • This work aims to add evidence and provide an update on the classification and diagnosis of monoclonal immunoglobulin deposition disease (MIDD) and primary central nervous system low-grade lymphomas. (biomedcentral.com)
  • The authors report unusual, generalized subretinal deposits and their course, documented by multimodal imaging, paralleling the systemic illness chronology in a patient with light chain deposition disease. (lww.com)
  • An important gene associated with Immunoglobulin Kappa Light Chain Deficiency is IGKC (Immunoglobulin Kappa Constant). (malacards.org)
  • Plasma cell dyscrasias may be responsible for Fanconi's syndrome, due to the toxicity of a free monoclonal kappa light chain toward kidney proximal tubules. (elsevier.com)
  • The amount of light chain that stimulated an intracellular redox-signaling pathway in the proximal tubule cells was well within levels that are seen in patients who have plasma cell dyscrasias. (asnjournals.org)
  • In the setting of a plasma cell dyscrasia, however, light chain production increases significantly, producing circulating concentrations of monoclonal light chain that can approach 100,000 mg/L ( 7 , 14 ). (asnjournals.org)
  • Mesangial injury represents a crucial event in the pathogenesis of light chain-associated glomerulopathies in patients with plasma cell dyscrasias. (heraldopenaccess.us)
  • If the heavy chain is able to bind to a surrogate light chain and move to the plasma membrane, then the developing B cell can begin producing its light chain. (wikipedia.org)