Alpha mannosidase. Medical search

Glycoside hydrolases that catalyze the hydrolysis of alpha or beta linked MANNOSE.

An enzyme that catalyzes the HYDROLYSIS of terminal, non-reducing alpha-D-mannose residues in alpha-D-mannosides. The enzyme plays a role in the processing of newly formed N-glycans and in degradation of mature GLYCOPROTEINS. There are multiple isoforms of alpha-mannosidase, each having its own specific cellular location and pH optimum. Defects in the lysosomal form of the enzyme results in a buildup of mannoside intermediate metabolites and the disease ALPHA-MANNOSIDOSIS.

An indolizidine alkaloid from the plant Swainsona canescens that is a potent alpha-mannosidase inhibitor. Swainsonine also exhibits antimetastatic, antiproliferative, and immunomodulatory activity.

An alpha-glucosidase inhibitor with antiviral action. Derivatives of deoxynojirimycin may have anti-HIV activity.

Carbohydrates consisting of between two (DISACCHARIDES) and ten MONOSACCHARIDES connected by either an alpha- or beta-glycosidic link. They are found throughout nature in both the free and bound form.

A hexose or fermentable monosaccharide and isomer of glucose from manna, the ash Fraxinus ornus and related plants. (From Grant & Hackh's Chemical Dictionary, 5th ed & Random House Unabridged Dictionary, 2d ed)

A stack of flattened vesicles that functions in posttranslational processing and sorting of proteins, receiving them from the rough ENDOPLASMIC RETICULUM and directing them to secretory vesicles, LYSOSOMES, or the CELL MEMBRANE. The movement of proteins takes place by transfer vesicles that bud off from the rough endoplasmic reticulum or Golgi apparatus and fuse with the Golgi, lysosomes or cell membrane. (From Glick, Glossary of Biochemistry and Molecular Biology, 1990)

Organic nitrogenous bases. Many alkaloids of medical importance occur in the animal and vegetable kingdoms, and some have been synthesized. (Grant & Hackh's Chemical Dictionary, 5th ed)

Diseases caused by the loss of one or more enzymes involved in the hydrolysis of mannoside linkages (MANNOSIDASES). The defects in enzyme activity are primarily associated with genetic mutation of the genes that codes for a particular mannosidase isoenzyme.

Plasma glycoprotein member of the serpin superfamily which inhibits TRYPSIN; NEUTROPHIL ELASTASE; and other PROTEOLYTIC ENZYMES.

Five-carbon furanose sugars in which the OXYGEN is replaced by a NITROGEN atom.

A system of cisternae in the CYTOPLASM of many cells. In places the endoplasmic reticulum is continuous with the plasma membrane (CELL MEMBRANE) or outer membrane of the nuclear envelope. If the outer surfaces of the endoplasmic reticulum membranes are coated with ribosomes, the endoplasmic reticulum is said to be rough-surfaced (ENDOPLASMIC RETICULUM, ROUGH); otherwise it is said to be smooth-surfaced (ENDOPLASMIC RETICULUM, SMOOTH). (King & Stansfield, A Dictionary of Genetics, 4th ed)

The sequence of carbohydrates within POLYSACCHARIDES; GLYCOPROTEINS; and GLYCOLIPIDS.

A group of related enzymes responsible for the endohydrolysis of the di-N-acetylchitobiosyl unit in high-mannose-content glycopeptides and GLYCOPROTEINS.

The chemical or biochemical addition of carbohydrate or glycosyl groups to other chemicals, especially peptides or proteins. Glycosyl transferases are used in this biochemical reaction.

Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.

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.

The characteristic 3-dimensional shape of a carbohydrate.

One of the two major pharmacological subdivisions of adrenergic receptors that were originally defined by the relative potencies of various adrenergic compounds. The alpha receptors were initially described as excitatory receptors that post-junctionally stimulate SMOOTH MUSCLE contraction. However, further analysis has revealed a more complex picture involving several alpha receptor subtypes and their involvement in feedback regulation.

Yeast-like ascomycetous fungi of the family Saccharomycetaceae, order SACCHAROMYCETALES isolated from exuded tree sap.

Hypoxia-inducible factor 1, alpha subunit is a basic helix-loop-helix transcription factor that is regulated by OXYGEN availability and is targeted for degradation by VHL TUMOR SUPPRESSOR PROTEIN.

A non-essential amino acid that is involved in the metabolic control of cell functions in nerve and brain tissue. It is biosynthesized from ASPARTIC ACID and AMMONIA by asparagine synthetase. (From Concise Encyclopedia Biochemistry and Molecular Biology, 3rd ed)

A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.

A fungal metabolite which is a macrocyclic lactone exhibiting a wide range of antibiotic activity.

The rate dynamics in chemical or physical systems.

The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.

Enzymes that catalyze the exohydrolysis of 1,4-alpha-glucosidic linkages with release of alpha-glucose. Deficiency of alpha-1,4-glucosidase may cause GLYCOGEN STORAGE DISEASE TYPE II.

Enzymes that catalyze the hydrolysis of N-acylhexosamine residues in N-acylhexosamides. Hexosaminidases also act on GLUCOSIDES; GALACTOSIDES; and several OLIGOSACCHARIDES.

Any of various enzymatically catalyzed post-translational modifications of PEPTIDES or PROTEINS in the cell of origin. These modifications include carboxylation; HYDROXYLATION; ACETYLATION; PHOSPHORYLATION; METHYLATION; GLYCOSYLATION; ubiquitination; oxidation; proteolysis; and crosslinking and result in changes in molecular weight and electrophoretic motility.

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.

Enzymes that catalyze the transfer of N-acetylglucosamine from a nucleoside diphosphate N-acetylglucosamine to an acceptor molecule which is frequently another carbohydrate. EC 2.4.1.-.

A beta-N-Acetylhexosaminidase that catalyzes the hydrolysis of terminal, non-reducing 2-acetamido-2-deoxy-beta-glucose residues in chitobiose and higher analogs as well as in glycoproteins. Has been used widely in structural studies on bacterial cell walls and in the study of diseases such as MUCOLIPIDOSIS and various inflammatory disorders of muscle and connective tissue.

An antiprotozoal agent produced by Streptomyces cinnamonensis. It exerts its effect during the development of first-generation trophozoites into first-generation schizonts within the intestinal epithelial cells. It does not interfere with hosts' development of acquired immunity to the majority of coccidial species. Monensin is a sodium and proton selective ionophore and is widely used as such in biochemical studies.

A member of the NICOTINIC ACETYLCHOLINE RECEPTOR subfamily of the LIGAND-GATED ION CHANNEL family. It consists entirely of pentameric a7 subunits expressed in the CNS, autonomic nervous system, vascular system, lymphocytes and spleen.

Techniques for removal by adsorption and subsequent elution of a specific antibody or antigen using an immunosorbent containing the homologous antigen or antibody.

An organic mercurial used as a sulfhydryl reagent.

Proteins prepared by recombinant DNA technology.

Enzymes that hydrolyze O-glucosyl-compounds. (Enzyme Nomenclature, 1992) EC 3.2.1.-.

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.

A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.

Cell surface receptor for LAMININ, epiligrin, FIBRONECTINS, entactin, and COLLAGEN. Integrin alpha3beta1 is the major integrin present in EPITHELIAL CELLS, where it plays a role in the assembly of BASEMENT MEMBRANE as well as in cell migration, and may regulate the functions of other integrins. Two alternatively spliced isoforms of the alpha subunit (INTEGRIN ALPHA3), are differentially expressed in different cell types.

An integrin alpha subunit that is unique in that it does not contain an I domain, and its proteolytic cleavage site is near the middle of the extracellular portion of the polypeptide rather than close to the membrane as in other integrin alpha subunits.

An integrin alpha subunit that primarily associates with INTEGRIN BETA1 or INTEGRIN BETA4 to form laminin-binding heterodimers. Integrin alpha6 has two alternatively spliced isoforms: integrin alpha6A and integrin alpha6B, which differ in their cytoplasmic domains and are regulated in a tissue-specific and developmental stage-specific manner.

An integrin found in FIBROBLASTS; PLATELETS; MONOCYTES, and LYMPHOCYTES. Integrin alpha5beta1 is the classical receptor for FIBRONECTIN, but it also functions as a receptor for LAMININ and several other EXTRACELLULAR MATRIX PROTEINS.

A subfamily in the family MURIDAE, comprising the hamsters. Four of the more common genera are Cricetus, CRICETULUS; MESOCRICETUS; and PHODOPUS.

Established cell cultures that have the potential to propagate indefinitely.

Single-stranded complementary DNA synthesized from an RNA template by the action of RNA-dependent DNA polymerase. cDNA (i.e., complementary DNA, not circular DNA, not C-DNA) is used in a variety of molecular cloning experiments as well as serving as a specific hybridization probe.

The N-acetyl derivative of glucosamine.

Thin structures that encapsulate subcellular structures or ORGANELLES in EUKARYOTIC CELLS. They include a variety of membranes associated with the CELL NUCLEUS; the MITOCHONDRIA; the GOLGI APPARATUS; the ENDOPLASMIC RETICULUM; LYSOSOMES; PLASTIDS; and VACUOLES.

Techniques used to separate mixtures of substances based on differences in the relative affinities of the substances for mobile and stationary phases. A mobile phase (fluid or gas) passes through a column containing a stationary phase of porous solid or liquid coated on a solid support. Usage is both analytical for small amounts and preparative for bulk amounts.

Integrin alpha4beta1 is a FIBRONECTIN and VCAM-1 receptor present on LYMPHOCYTES; MONOCYTES; EOSINOPHILS; NK CELLS and thymocytes. It is involved in both cell-cell and cell- EXTRACELLULAR MATRIX adhesion and plays a role in INFLAMMATION, hematopoietic cell homing and immune function, and has been implicated in skeletal MYOGENESIS; NEURAL CREST migration and proliferation, lymphocyte maturation and morphogenesis of the PLACENTA and HEART.

A protein complex comprised of COATOMER PROTEIN and ADP RIBOSYLATION FACTOR 1. It is involved in transport of vesicles between the ENDOPLASMIC RETICULUM and the GOLGI APPARATUS.

An interleukin-1 subtype that occurs as a membrane-bound pro-protein form that is cleaved by proteases to form a secreted mature form. Unlike INTERLEUKIN-1BETA both membrane-bound and secreted forms of interleukin-1alpha are biologically active.

The uptake of naked or purified DNA by CELLS, usually meaning the process as it occurs in eukaryotic cells. It is analogous to bacterial transformation (TRANSFORMATION, BACTERIAL) and both are routinely employed in GENE TRANSFER TECHNIQUES.

An integrin found on fibroblasts, platelets, endothelial and epithelial cells, and lymphocytes where it functions as a receptor for COLLAGEN and LAMININ. Although originally referred to as the collagen receptor, it is one of several receptors for collagen. Ligand binding to integrin alpha2beta1 triggers a cascade of intracellular signaling, including activation of p38 MAP kinase.

Enzymes that catalyze the transfer of galactose from a nucleoside diphosphate galactose to an acceptor molecule which is frequently another carbohydrate. EC 2.4.1.-.

The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.

A subclass of alpha-adrenergic receptors that mediate contraction of SMOOTH MUSCLE in a variety of tissues such as ARTERIOLES; VEINS; and the UTERUS. They are usually found on postsynaptic membranes and signal through GQ-G11 G-PROTEINS.

This integrin alpha subunit combines with INTEGRIN BETA1 to form a receptor (INTEGRIN ALPHA5BETA1) that binds FIBRONECTIN and LAMININ. It undergoes posttranslational cleavage into a heavy and a light chain that are connected by disulfide bonds.

Integrin alpha1beta1 functions as a receptor for LAMININ and COLLAGEN. It is widely expressed during development, but in the adult is the predominant laminin receptor (RECEPTORS, LAMININ) in mature SMOOTH MUSCLE CELLS, where it is important for maintenance of the differentiated phenotype of these cells. Integrin alpha1beta1 is also found in LYMPHOCYTES and microvascular endothelial cells, and may play a role in angiogenesis. In SCHWANN CELLS and neural crest cells, it is involved in cell migration. Integrin alpha1beta1 is also known as VLA-1 and CD49a-CD29.

Polysaccharides consisting of mannose units.

Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors.

A subclass of alpha-adrenergic receptors found on both presynaptic and postsynaptic membranes where they signal through Gi-Go G-PROTEINS. While postsynaptic alpha-2 receptors play a traditional role in mediating the effects of ADRENERGIC AGONISTS, the subset of alpha-2 receptors found on presynaptic membranes signal the feedback inhibition of NEUROTRANSMITTER release.

The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.

An N-acetylglycosamine containing antiviral antibiotic obtained from Streptomyces lysosuperificus. It is also active against some bacteria and fungi, because it inhibits the glucosylation of proteins. Tunicamycin is used as tool in the study of microbial biosynthetic mechanisms.

A cell surface receptor mediating cell adhesion to the EXTRACELLULAR MATRIX and to other cells via binding to LAMININ. It is involved in cell migration, embryonic development, leukocyte activation and tumor cell invasiveness. Integrin alpha6beta1 is the major laminin receptor on PLATELETS; LEUKOCYTES; and many EPITHELIAL CELLS, and ligand binding may activate a number of signal transduction pathways. Alternative splicing of the cytoplasmic domain of the alpha6 subunit (INTEGRIN ALPHA6) results in the formation of A and B isoforms of the heterodimer, which are expressed in a tissue-specific manner.

The parts of a macromolecule that directly participate in its specific combination with another molecule.

The process of cleaving a chemical compound by the addition of a molecule of water.

A partitioning within cells due to the selectively permeable membranes which enclose each of the separate parts, e.g., mitochondria, lysosomes, etc.

Compounds or agents that combine with an enzyme in such a manner as to prevent the normal substrate-enzyme combination and the catalytic reaction.

CELL LINE derived from the ovary of the Chinese hamster, Cricetulus griseus (CRICETULUS). The species is a favorite for cytogenetic studies because of its small chromosome number. The cell line has provided model systems for the study of genetic alterations in cultured mammalian cells.

This intrgrin is a key component of HEMIDESMOSOMES and is required for their formation and maintenance in epithelial cells. Integrin alpha6beta4 is also found on thymocytes, fibroblasts, and Schwann cells, where it functions as a laminin receptor (RECEPTORS, LAMININ) and is involved in wound healing, cell migration, and tumor invasiveness.

The alpha subunits of integrin heterodimers (INTEGRINS), which mediate ligand specificity. There are approximately 18 different alpha chains, exhibiting great sequence diversity; several chains are also spliced into alternative isoforms. They possess a long extracellular portion (1200 amino acids) containing a MIDAS (metal ion-dependent adhesion site) motif, and seven 60-amino acid tandem repeats, the last 4 of which form EF HAND MOTIFS. The intracellular portion is short with the exception of INTEGRIN ALPHA4.

Closed vesicles of fragmented endoplasmic reticulum created when liver cells or tissue are disrupted by homogenization. They may be smooth or rough.

A family of transmembrane glycoproteins (MEMBRANE GLYCOPROTEINS) consisting of noncovalent heterodimers. They interact with a wide variety of ligands including EXTRACELLULAR MATRIX PROTEINS; COMPLEMENT, and other cells, while their intracellular domains interact with the CYTOSKELETON. The integrins consist of at least three identified families: the cytoadhesin receptors(RECEPTORS, CYTOADHESIN), the leukocyte adhesion receptors (RECEPTORS, LEUKOCYTE ADHESION), and the VERY LATE ANTIGEN RECEPTORS. Each family contains a common beta-subunit (INTEGRIN BETA CHAINS) combined with one or more distinct alpha-subunits (INTEGRIN ALPHA CHAINS). These receptors participate in cell-matrix and cell-cell adhesion in many physiologically important processes, including embryological development; HEMOSTASIS; THROMBOSIS; WOUND HEALING; immune and nonimmune defense mechanisms; and oncogenic transformation.

An integrin alpha subunit that binds COLLAGEN and LAMININ though its I domain. It combines with INTEGRIN BETA1 to form the heterodimer INTEGRIN ALPHA1BETA1.

Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.

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 group of alicyclic hydrocarbons with the general formula R-C5H9.

Enzymes that catalyze the transfer of mannose from a nucleoside diphosphate mannose to an acceptor molecule which is frequently another carbohydrate. The group includes EC 2.4.1.32, EC 2.4.1.48, EC 2.4.1.54, and EC 2.4.1.57.

Brain waves characterized by a relatively high voltage or amplitude and a frequency of 8-13 Hz. They constitute the majority of waves recorded by EEG registering the activity of the parietal and occipital lobes when the individual is awake, but relaxed with the eyes closed.

Processes involved in the formation of TERTIARY PROTEIN STRUCTURE.

Genetically identical individuals developed from brother and sister matings which have been carried out for twenty or more generations or by parent x offspring matings carried out with certain restrictions. This also includes animals with a long history of closed colony breeding.

The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.

Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy.

A large multisubunit complex that plays an important role in the degradation of most of the cytosolic and nuclear proteins in eukaryotic cells. It contains a 700-kDa catalytic sub-complex and two 700-kDa regulatory sub-complexes. The complex digests ubiquitinated proteins and protein activated via ornithine decarboxylase antizyme.

An integrin alpha subunit that occurs as alternatively spliced isoforms. The isoforms are differentially expressed in specific cell types and at specific developmental stages. Integrin alpha3 combines with INTEGRIN BETA1 to form INTEGRIN ALPHA3BETA1 which is a heterodimer found primarily in epithelial cells.

Deficiency of the protease inhibitor ALPHA 1-ANTITRYPSIN that manifests primarily as PULMONARY EMPHYSEMA and LIVER CIRRHOSIS.

Nonionic surfactant mixtures varying in the number of repeating ethoxy (oxy-1,2-ethanediyl) groups. They are used as detergents, emulsifiers, wetting agents, defoaming agents, etc. Octoxynol-9, the compound with 9 repeating ethoxy groups, is a spermatocide.

Glycoproteins found on the membrane or surface of cells.

One of the two major classes of cholinergic receptors. Nicotinic receptors were originally distinguished by their preference for NICOTINE over MUSCARINE. They are generally divided into muscle-type and neuronal-type (previously ganglionic) based on pharmacology, and subunit composition of the receptors.

Drugs that selectively bind to and activate alpha adrenergic receptors.

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.

Techniques to partition various components of the cell into SUBCELLULAR FRACTIONS.

Recombinant proteins produced by the GENETIC TRANSLATION of fused genes formed by the combination of NUCLEIC ACID REGULATORY SEQUENCES of one or more genes with the protein coding sequences of one or more genes.

The process of moving proteins from one cellular compartment (including extracellular) to another by various sorting and transport mechanisms such as gated transport, protein translocation, and vesicular transport.

The sum of the weight of all the atoms in a molecule.

Positively charged atoms, radicals or groups of atoms with a valence of plus 2, which travel to the cathode or negative pole during electrolysis.

A class of morphologically heterogeneous cytoplasmic particles in animal and plant tissues characterized by their content of hydrolytic enzymes and the structure-linked latency of these enzymes. The intracellular functions of lysosomes depend on their lytic potential. The single unit membrane of the lysosome acts as a barrier between the enzymes enclosed in the lysosome and the external substrate. The activity of the enzymes contained in lysosomes is limited or nil unless the vesicle in which they are enclosed is ruptured. Such rupture is supposed to be under metabolic (hormonal) control. (From Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed)

The phenotypic manifestation of a gene or genes by the processes of GENETIC TRANSCRIPTION and GENETIC TRANSLATION.

A nuclear transcription factor. Heterodimerization with RETINOID X RECEPTOR GAMMA is important to metabolism of LIPIDS. It is the target of FIBRATES to control HYPERLIPIDEMIAS.

A naturally occurring prostaglandin that has oxytocic, luteolytic, and abortifacient activities. Due to its vasocontractile properties, the compound has a variety of other biological actions.

Intracellular fluid from the cytoplasm after removal of ORGANELLES and other insoluble cytoplasmic components.

Cells grown in vitro from neoplastic tissue. If they can be established as a TUMOR CELL LINE, they can be propagated in cell culture indefinitely.

The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells.

The relationship between the chemical structure of a compound and its biological or pharmacological activity. Compounds are often classed together because they have structural characteristics in common including shape, size, stereochemical arrangement, and distribution of functional groups.

Short sequences (generally about 10 base pairs) of DNA that are complementary to sequences of messenger RNA and allow reverse transcriptases to start copying the adjacent sequences of mRNA. Primers are used extensively in genetic and molecular biology techniques.

Drugs that bind to but do not activate alpha-adrenergic receptors thereby blocking the actions of endogenous or exogenous adrenergic agonists. Adrenergic alpha-antagonists are used in the treatment of hypertension, vasospasm, peripheral vascular disease, shock, and pheochromocytoma.

Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.

Proteins which contain carbohydrate groups attached covalently to the polypeptide chain. The protein moiety is the predominant group with the carbohydrate making up only a small percentage of the total weight.

Structurally related forms of an enzyme. Each isoenzyme has the same mechanism and classification, but differs in its chemical, physical, or immunological characteristics.

Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.

A species of the genus SACCHAROMYCES, family Saccharomycetaceae, order Saccharomycetales, known as "baker's" or "brewer's" yeast. The dried form is used as a dietary supplement.

Body organ that filters blood for the secretion of URINE and that regulates ion concentrations.

Any of various animals that constitute the family Suidae and comprise stout-bodied, short-legged omnivorous mammals with thick skin, usually covered with coarse bristles, a rather long mobile snout, and small tail. Included are the genera Babyrousa, Phacochoerus (wart hogs), and Sus, the latter containing the domestic pig (see SUS SCROFA).

Hepatocyte nuclear factor 1-alpha is a transcription factor found in the LIVER; PANCREAS; and KIDNEY that regulates HOMEOSTASIS of GLUCOSE.

The first continuously cultured human malignant CELL LINE, derived from the cervical carcinoma of Henrietta Lacks. These cells are used for VIRUS CULTIVATION and antitumor drug screening assays.

The normality of a solution with respect to HYDROGEN ions; H+. It is related to acidity measurements in most cases by pH = log 1/2[1/(H+)], where (H+) is the hydrogen ion concentration in gram equivalents per liter of solution. (McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed)

Glycoprotein found in alpha(1)-globulin region in human serum. It inhibits chymotrypsin-like proteinases in vivo and has cytotoxic killer-cell activity in vitro. The protein also has a role as an acute-phase protein and is active in the control of immunologic and inflammatory processes, and as a tumor marker. It is a member of the serpin superfamily.

The movement of materials (including biochemical substances and drugs) through a biological system at the cellular level. The transport can be across cell membranes and epithelial layers. It also can occur within intracellular compartments and extracellular compartments.

An EPIDERMAL GROWTH FACTOR related protein that is found in a variety of tissues including EPITHELIUM, and maternal DECIDUA. It is synthesized as a transmembrane protein which can be cleaved to release a soluble active form which binds to the EGF RECEPTOR.

The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.

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.

Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control (induction or repression) of gene action at the level of transcription or translation.

Liquid chromatographic techniques which feature high inlet pressures, high sensitivity, and high speed.

Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.

Endogenous substances, usually proteins, which are effective in the initiation, stimulation, or termination of the genetic transcription process.

Drugs that bind to and block the activation of ADRENERGIC ALPHA-1 RECEPTORS.

Nucleocytoplasmic transport molecules that bind to the NUCLEAR LOCALIZATION SIGNALS of cytoplasmic molecules destined to be imported into the CELL NUCLEUS. Once attached to their cargo they bind to BETA KARYOPHERINS and are transported through the NUCLEAR PORE COMPLEX. Inside the CELL NUCLEUS alpha karyopherins dissociate from beta karypherins and their cargo. They then form a complex with CELLULAR APOPTOSIS SUSCEPTIBILITY PROTEIN and RAN GTP-BINDING PROTEIN which is exported to the CYTOPLASM.

Systems of enzymes which function sequentially by catalyzing consecutive reactions linked by common metabolic intermediates. They may involve simply a transfer of water molecules or hydrogen atoms and may be associated with large supramolecular structures such as MITOCHONDRIA or RIBOSOMES.

Compounds and molecular complexes that consist of very large numbers of atoms and are generally over 500 kDa in size. In biological systems macromolecular substances usually can be visualized using ELECTRON MICROSCOPY and are distinguished from ORGANELLES by the lack of a membrane structure.

Serum glycoprotein produced by activated MACROPHAGES and other mammalian MONONUCLEAR LEUKOCYTES. It has necrotizing activity against tumor cell lines and increases ability to reject tumor transplants. Also known as TNF-alpha, it is only 30% homologous to TNF-beta (LYMPHOTOXIN), but they share TNF RECEPTORS.

Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules.

A selective adrenergic alpha-1 antagonist used in the treatment of HEART FAILURE; HYPERTENSION; PHEOCHROMOCYTOMA; RAYNAUD DISEASE; PROSTATIC HYPERTROPHY; and URINARY RETENTION.

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.

The ability of a substance to be dissolved, i.e. to form a solution with another substance. (From McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed)

A genus of owlet moths of the family Noctuidae. These insects are used in molecular biology studies during all stages of their life cycle.

The physiologically active and stable hydrolysis product of EPOPROSTENOL. Found in nearly all mammalian tissue.

Single chains of amino acids that are the units of multimeric PROTEINS. Multimeric proteins can be composed of identical or non-identical subunits. One or more monomeric subunits may compose a protomer which itself is a subunit structure of a larger assembly.

Chemical groups containing the covalent disulfide bonds -S-S-. The sulfur atoms can be bound to inorganic or organic moieties.

Separation technique in which the stationary phase consists of ion exchange resins. The resins contain loosely held small ions that easily exchange places with other small ions of like charge present in solutions washed over the resins.

The relationship between the dose of an administered drug and the response of the organism to the drug.

A large collection of DNA fragments cloned (CLONING, MOLECULAR) from a given organism, tissue, organ, or cell type. It may contain complete genomic sequences (GENOMIC LIBRARY) or complementary DNA sequences, the latter being formed from messenger RNA and lacking intron sequences.

Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.

The biosynthesis of RNA carried out on a template of DNA. The biosynthesis of DNA from an RNA template is called REVERSE TRANSCRIPTION.

Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen.

Regulatory proteins that act as molecular switches. They control a wide range of biological processes including: receptor signaling, intracellular signal transduction pathways, and protein synthesis. Their activity is regulated by factors that control their ability to bind to and hydrolyze GTP to GDP. EC 3.6.1.-.

The domestic dog, Canis familiaris, comprising about 400 breeds, of the carnivore family CANIDAE. They are worldwide in distribution and live in association with people. (Walker's Mammals of the World, 5th ed, p1065)

Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.

The introduction of a phosphoryl group into a compound through the formation of an ester bond between the compound and a phosphorus moiety.

An imidazoline sympatholytic agent that stimulates ALPHA-2 ADRENERGIC RECEPTORS and central IMIDAZOLINE RECEPTORS. It is commonly used in the management of HYPERTENSION.

A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of SKIN; CONNECTIVE TISSUE; and the organic substance of bones (BONE AND BONES) and teeth (TOOTH).

DNA sequences which are recognized (directly or indirectly) and bound by a DNA-dependent RNA polymerase during the initiation of transcription. Highly conserved sequences within the promoter include the Pribnow box in bacteria and the TATA BOX in eukaryotes.

Layers of protein which surround the capsid in animal viruses with tubular nucleocapsids. The envelope consists of an inner layer of lipids and virus specified proteins also called membrane or matrix proteins. The outer layer consists of one or more types of morphological subunits called peplomers which project from the viral envelope; this layer always consists of glycoproteins.

A plant alkaloid with alpha-2-adrenergic blocking activity. Yohimbine has been used as a mydriatic and in the treatment of ERECTILE DYSFUNCTION.

A mass spectrometric technique that is used for the analysis of large biomolecules. Analyte molecules are embedded in an excess matrix of small organic molecules that show a high resonant absorption at the laser wavelength used. The matrix absorbs the laser energy, thus inducing a soft disintegration of the sample-matrix mixture into free (gas phase) matrix and analyte molecules and molecular ions. In general, only molecular ions of the analyte molecules are produced, and almost no fragmentation occurs. This makes the method well suited for molecular weight determinations and mixture analysis.

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).

Proteins which bind to DNA. The family includes proteins which bind to both double- and single-stranded DNA and also includes specific DNA binding proteins in serum which can be used as markers for malignant diseases.

ENDOPEPTIDASES which have a cysteine involved in the catalytic process. This group of enzymes is inactivated by CYSTEINE PROTEINASE INHIBITORS such as CYSTATINS and SULFHYDRYL REAGENTS.

A soluble factor produced by MONOCYTES; MACROPHAGES, and other cells which activates T-lymphocytes and potentiates their response to mitogens or antigens. Interleukin-1 is a general term refers to either of the two distinct proteins, INTERLEUKIN-1ALPHA and INTERLEUKIN-1BETA. The biological effects of IL-1 include the ability to replace macrophage requirements for T-cell activation.

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).

Compounds that bind to and activate ADRENERGIC ALPHA-2 RECEPTORS.

A strain of albino rat used widely for experimental purposes because of its calmness and ease of handling. It was developed by the Sprague-Dawley Animal Company.

A family of heterotrimeric GTP-binding protein alpha subunits that were originally identified by their ability to inhibit ADENYLYL CYCLASES. Members of this family can couple to beta and gamma G-protein subunits that activate POTASSIUM CHANNELS. The Gi-Go part of the name is also spelled Gi/Go.

A variation of the PCR technique in which cDNA is made from RNA via reverse transcription. The resultant cDNA is then amplified using standard PCR protocols.

A molecule that binds to another molecule, used especially to refer to a small molecule that binds specifically to a larger molecule, e.g., an antigen binding to an antibody, a hormone or neurotransmitter binding to a receptor, or a substrate or allosteric effector binding to an enzyme. Ligands are also molecules that donate or accept a pair of electrons to form a coordinate covalent bond with the central metal atom of a coordination complex. (From Dorland, 27th ed)

Different forms of a protein that may be produced from different GENES, or from the same gene by ALTERNATIVE SPLICING.

Drugs that bind to and block the activation of ADRENERGIC ALPHA-2 RECEPTORS.

Elements of limited time intervals, contributing to particular results or situations.

Peptide elongation factor 1 is a multisubunit protein that is responsible for the GTP-dependent binding of aminoacyl-tRNAs to eukaryotic ribosomes. The alpha subunit (EF-1alpha) binds aminoacyl-tRNA and transfers it to the ribosome in a process linked to GTP hydrolysis. The beta and delta subunits (EF-1beta, EF-1delta) are involved in exchanging GDP for GTP. The gamma subunit (EF-1gamma) is a structural component.

A cell line derived from cultured tumor cells.

A subfamily of nuclear receptors that regulate GENETIC TRANSCRIPTION of a diverse group of GENES involved in the synthesis of BLOOD COAGULATION FACTORS; and in GLUCOSE; CHOLESTEROL; and FATTY ACIDS metabolism.

Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.

Cell surface proteins which bind GAMMA-AMINOBUTYRIC ACID and contain an integral membrane chloride channel. Each receptor is assembled as a pentamer from a pool of at least 19 different possible subunits. The receptors belong to a superfamily that share a common CYSTEINE loop.

(9 alpha,11 alpha,13E,15S)-9,11,15-Trihydroxyprost-13-en-1-oic acid (PGF(1 alpha)); (5Z,9 alpha,11,alpha,13E,15S)-9,11,15-trihydroxyprosta-5,13-dien-1-oic acid (PGF(2 alpha)); (5Z,9 alpha,11 alpha,13E,15S,17Z)-9,11,15-trihydroxyprosta-5,13,17-trien-1-oic acid (PGF(3 alpha)). A family of prostaglandins that includes three of the six naturally occurring prostaglandins. All naturally occurring PGF have an alpha configuration at the 9-carbon position. They stimulate uterine and bronchial smooth muscle and are often used as oxytocics.

Histochemical localization of immunoreactive substances using labeled antibodies as reagents.

Conversion of an inactive form of an enzyme to one possessing metabolic activity. It includes 1, activation by ions (activators); 2, activation by cofactors (coenzymes); and 3, conversion of an enzyme precursor (proenzyme or zymogen) to an active enzyme.

Genetically engineered MUTAGENESIS at a specific site in the DNA molecule that introduces a base substitution, or an insertion or deletion.

A condition of decreased oxygen content at the cellular level.

Drugs that bind to nicotinic cholinergic receptors (RECEPTORS, NICOTINIC) and block the actions of acetylcholine or cholinergic agonists. Nicotinic antagonists block synaptic transmission at autonomic ganglia, the skeletal neuromuscular junction, and at central nervous system nicotinic synapses.

Antibodies produced by a single clone of cells.

The interaction of two or more substrates or ligands with the same binding site. The displacement of one by the other is used in quantitative and selective affinity measurements.

A family of heterotrimeric GTP-binding protein alpha subunits that activate ADENYLYL CYCLASES.

A positive regulatory effect on physiological processes at the molecular, cellular, or systemic level. At the molecular level, the major regulatory sites include membrane receptors, genes (GENE EXPRESSION REGULATION), mRNAs (RNA, MESSENGER), and proteins.

Drugs that bind to and activate nicotinic cholinergic receptors (RECEPTORS, NICOTINIC). Nicotinic agonists act at postganglionic nicotinic receptors, at neuroeffector junctions in the peripheral nervous system, and at nicotinic receptors in the central nervous system. Agents that function as neuromuscular depolarizing blocking agents are included here because they activate nicotinic receptors, although they are used clinically to block nicotinic transmission.

Heparin-binding proteins that exhibit a number of inflammatory and immunoregulatory activities. Originally identified as secretory products of MACROPHAGES, these chemokines are produced by a variety of cell types including NEUTROPHILS; FIBROBLASTS; and EPITHELIAL CELLS. They likely play a significant role in respiratory tract defenses.

Specific cell surface receptors which bind to FIBRONECTINS. Studies have shown that these receptors function in certain types of adhesive contact as well as playing a major role in matrix assembly. These receptors include the traditional fibronectin receptor, also called INTEGRIN ALPHA5BETA1 and several other integrins.

A 38-kDa mitogen-activated protein kinase that is abundantly expressed in a broad variety of cell types. It is involved in the regulation of cellular stress responses as well as the control of proliferation and survival of many cell types. The kinase activity of the enzyme is inhibited by the pyridinyl-imidazole compound SB 203580.

Enzymes catalyzing the transfer of fucose from a nucleoside diphosphate fucose to an acceptor molecule which is frequently another carbohydrate, a glycoprotein, or a glycolipid molecule. Elevated activity of some fucosyltransferases in human serum may serve as an indicator of malignancy. The class includes EC 2.4.1.65; EC 2.4.1.68; EC 2.4.1.69; EC 2.4.1.89.

An enzyme that catalyzes the active transport system of sodium and potassium ions across the cell wall. Sodium and potassium ions are closely coupled with membrane ATPase which undergoes phosphorylation and dephosphorylation, thereby providing energy for transport of these ions against concentration gradients.

A family of heterotrimeric GTP-binding protein alpha subunits that activate TYPE C PHOSPHOLIPASES dependent signaling pathways. The Gq-G11 part of the name is also spelled Gq/G11.

Serum proteins that have the most rapid migration during ELECTROPHORESIS. This subgroup of globulins is divided into faster and slower alpha(1)- and alpha(2)-globulins.

Differentiation antigens residing on mammalian leukocytes. CD stands for cluster of differentiation, which refers to groups of monoclonal antibodies that show similar reactivity with certain subpopulations of antigens of a particular lineage or differentiation stage. The subpopulations of antigens are also known by the same CD designation.

Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.

The GTPase-containing subunits of heterotrimeric GTP-binding proteins. When dissociated from the heterotrimeric complex these subunits interact with a variety of second messenger systems. Hydrolysis of GTP by the inherent GTPase activity of the subunit causes it to revert to its inactive (heterotrimeric) form. The GTP-Binding protein alpha subunits are grouped into families according to the type of action they have on second messenger systems.

Glycoproteins found on the surfaces of cells, particularly in fibrillar structures. The proteins are lost or reduced when these cells undergo viral or chemical transformation. They are highly susceptible to proteolysis and are substrates for activated blood coagulation factor VIII. The forms present in plasma are called cold-insoluble globulins.

The movement of cells from one location to another. Distinguish from CYTOKINESIS which is the process of dividing the CYTOPLASM of a cell.

Endometrial lysosomal enzyme activity in normal cycling endometrium. (1/346)

The objective of this study was to evaluate the possible role of four lysosomal enzymes in endometrial function and remodelling during the normal menstrual cycle by fluorimetric measurement (acid phosphatase, N-acetyl-beta-D-glucosaminidase, alpha-L-fucosidase and alpha-D-mannosidase). A prospective study was conducted of 45 endometrial biopsies obtained from women with normal menstrual cycles. Activity of all four enzymes was identified in human endometrium. Activity of acid phosphatase and N-acetyl-beta-D-glucosaminidase was relatively high, whilst that of alpha-L-fucosidase and alpha-D-mannosidase was low. There was no significant change in the activity of any of the four enzymes from the proliferative to the secretory phase of the cycle. This study suggests that the activity of these enzymes remains constant throughout a major portion of the normal cycle. (+info)

Mid2 is a putative sensor for cell integrity signaling in Saccharomyces cerevisiae. (2/346)

Hcs77 is a putative cell surface sensor for cell integrity signaling in Saccharomyces cerevisiae. Its loss of function results in cell lysis during growth at elevated temperatures (e.g., 39 degrees C) and impaired signaling to the Mpk1 mitogen-activated protein kinase in response to mild heat shock. We isolated the MID2 gene as a dosage suppressor of the cell lysis defect of an hcs77 null mutant. MID2 encodes a putative membrane protein whose function is required for survival of pheromone treatment. Mid2 possesses properties similar to those of Hcs77, including a single transmembrane domain and a long region that is rich in seryl and threonyl residues. We demonstrate that Mid2 is required for cell integrity signaling in response to pheromone. Additionally, we show that Mid2 and Hcs77 serve a redundant but essential function as cell surface sensors for cell integrity signaling during vegetative growth. Both proteins are uniformly distributed through the plasma membrane and are highly O-mannosylated on their extracellular domains. Finally, we identified a yeast homolog of MID2, designated MTL1, which provides a partially redundant function with MID2 for cell integrity signaling during vegetative growth at elevated temperature but not for survival of pheromone treatment. We conclude that Hcs77 is dedicated to signaling cell wall stress during vegetative growth and that Mid2 participates in this signaling, but its primary role is in signaling wall stress during pheromone-induced morphogenesis. (+info)

Purification and biochemical characterization of two soluble alpha-mannosidases from Candida albicans. (3/346)

Two soluble alpha-mannosidases, E-I and E-II, were purified from C. albicans yeast cells by a three-step procedure consisting of size exclusion and ion exchange chromatographies in Sepharose CL6B and Mono Q columns, respectively, and preparative nondenaturing electrophoresis. E-I and E-II migrated as monomeric polypeptides of 54.3 and 93.3 kDa in SDS-PAGE, respectively. Some biochemical properties of purified enzymes were investigated by using 4-methylumbelliferyl-alpha-D-mannopyranoside and p-nitrophenyl-alpha-D-mannopyranoside as substrates. Hydrolysis of both substrates by either enzyme was optimum at pH 6.0 with 50 mM Mes-Tris buffer and at 42 degrees C. Apparent Kmvalues for hydrolysis of 4-methylumbelliferyl-alpha-D-mannopyranoside and p-nitrophenyl-alpha-D-mannopyranoside by E-I were 0.83 microM and 2. 4 mM, respectively. Corresponding values for E-II were 0.25 microM and 1.86 mM. Swansonine and deoxymannojirimicin strongly inhibited the hydrolysis of 4-methylumbelliferyl-alpha-D-mannopyranoside by both enzymes. On the contrary, hydrolysis of p-nitrophenyl-alpha-D-mannopyranoside by E-I and E-II was slightly stimulated or not affected, respectively, by both inhibitors. E-I and E-II did not depend on metal ions although activity of the latter was slightly stimulated by Mn2+and Ca2+in the range of 0.5-2 mM. At the same concentrations, Mg2+was slightly inhibitory of both enzymes. Substrate specificity experiments revealed that both E-I and E-II preferentially cleaved alpha-1,6 and alpha-1,3 linkages, respectively. (+info)

Characterization of a soluble class I alpha-mannosidase in human serum. (4/346)

Class I alpha-mannosidases are thought to exist exclusively as integral membrane proteins that play intracellulary an essential role in the N-glycan biosynthesis. Using [3H]Man9GlcNAc2 as a substrate, we were able to identify a soluble alpha-mannosidase in human serum that trims the substrate Man9GlcNAc2 to Man(5-8)GlcNAc2 with Man6GlcNAc2 being the major product. This serum mannosidase is Ca2+-dependent, sensitive to 1-deoxymannojirimycin but insensitive to the class II inhibitor swainsonine and, hence, belongs to class I mannosidases. The enzymatic properties of the serum class I mannosidase are similar to that of the membrane bound class I mannosidases Golgi-mannosidase IA and IB and Man9-mannosidase. (+info)

Androgen regulation of glycosidase secretion in epithelial cell cultures from human epididymis. (5/346)

The human epididymis and its secretions actively promote sperm fertilizing capacity and provide protection for spermatozoa against harmful influences. Among epididymal secretions, glycosidases have been recently studied and associated with molecular changes on the sperm surface. In the present work, we studied the influence of different concentrations of testosterone, dihydrotestosterone and cyproterone acetate on the secretion of alpha-glucosidase, N-acetyl-glucosaminidase, beta-glucuronidase and alpha-mannosidase by isolated and cultured epithelial cells from human caput, corpus and cauda epididymides. Cell cultures were obtained from aggregates of isolated tubule fragments plated on extracellular matrix-covered multi-well plates. Activities of the glycosidases were measured in conditioned culture media and were higher in the distal regions of the epididymis. Testosterone and dihydrotestosterone significantly increase the enzyme secretion in a concentration-dependent manner. This increase was higher in corpus and/or cauda than in caput epididymis. Cyproterone acetate caused a dose-dependent decrease in glycosidase secretion in cultures from all epididymal regions. It is concluded that the secretion of epididymal glycosidases is regulated by androgen, being stimulated by dihydrotestosterone and testosterone and inhibited by the androgen antagonist cyproterone acetate. (+info)

Topological and functional characterization of the N-glycans of soybean (Glycine max) agglutinin. (6/346)

Soybean agglutinin (SBA), is a noncovalently bound tetramer comprised of four identical subunits having a single N-glycan chain, Man9GlcNAc2, that is known to be essential for regeneration of the functional tetrameric structure from unfolded subunits. In this study, SBA was found to have strong affinity for concanavalin A, indicating that the N-glycans are extensively solvent-exposed. The susceptibilities of the N-glycans to alpha-mannosidase and endo-beta-N-acetylglucosaminidase revealed that their distal areas have nonreducing ends embedded among the subunits, whereas their proximal regions are solvent-exposed. Endo-beta-N-acetylglucosaminidase-digested SBA was unable to retain its conformation and gradually unfolded. Periodate-oxidized SBA, whose N-glycans closely correspond to the invariant pentasaccharide core, tended to dissociate into the subunits, but permitted to stay as folded monomers. This SBA species was capable of refolding from unfolded subunits but unable to form the functional tetramer. It seems probable that the proximal regions of the N-glycans function in the formation and stabilization of the subunit conformation, whereas the branches outside the invariant cores stabilize the tetrameric structure. (+info)

Targeted disruption of the lysosomal alpha-mannosidase gene results in mice resembling a mild form of human alpha-mannosidosis. (7/346)

Alpha-mannosidosis is a lysosomal storage disease with autosomal recessive inheritance caused by a deficiency of the lysosomal alpha-mannosidase, which is involved in the degradation of asparagine-linked carbohydrate cores of glycoproteins. An alpha-mannosidosis mouse model was generated by targeted disruption of the gene for lysosomal alpha-mannosidase. Homozygous mutant animals exhibit alpha-mannosidase enzyme deficiency and elevated urinary secretion of mannose-containing oligosaccharides. Thin-layer chromatography revealed an accumulation of oligosaccharides in liver, kidney, spleen, testis and brain. The cellular alterations were characterized by multiple membrane-limited cytoplasmic vacuoles as seen for instance in liver, exocrine pancreas, kidney, thyroid gland, smooth muscle cells, osteocytes and in various neurons of the central and peripheral nervous systems. The morphological lesions and their topographical distribution, as well as the biochemical alterations, closely resemble those reported for human alpha-mannosidosis. This mouse model will be a valuable tool for studying the pathogenesis of inherited alpha-mannosidosis and may help to evaluate therapeutic approaches for lysosomal storage diseases. (+info)

Purification and properties of major alpha-D-mannosidase in the luminal fluid of porcine epididymis. (8/346)

A lysosomal type alpha-D-mannosidase was successfully purified by DEAE-Sephacel, Red-Amicon and Superdex 200 column chromatographies from porcine cauda epididymal fluid. The purified enzyme consisted of 63 and 51 kDa subunits at equimolar amounts. It cleaved alpha1-2 linked mannosyl residues and less but significantly cleaved alpha1-3 and alpha1-6 linked mannosyl residues in the high-mannose oligosaccharides. The optimal pH to hydrolyze oligosaccharide was in the acidic pH range (pH 3.5 approximately 4.0). Total alpha-D-mannosidase activities in the porcine epididymal fluid increased from proximal to distal caput epididymis, which maintained to cauda epididymis. At least two kinds of alpha-D-mannosidase (lysosomal type enzyme and 135 kDa alpha-D-mannosidase (MAN2B2)) were contained in the porcine epididymal fluid. The activity of the lysosomal type enzyme is much higher than MAN2B2 at the physiological pH. These results suggest that the lysosomal type alpha-D-mannosidase is the predominantly active enzyme in the luminal fluid of porcine epididymis and that it participates in the glycoprotein modification on the sperm surface during epididymal transit. (+info)

... alpha-D-mannosidase, p-nitrophenyl-alpha-mannosidase, alpha-D-mannopyranosidase, 1,2-alpha-mannosidase, 1,2-alpha-D-mannosidase ... GeneReviews/NCBI/NIH/UW entry on Alpha-Mannosidosis OMIM entries on Alpha-Mannosidosis alpha-Mannosidase at the US National ... exo-alpha-mannosidase) is an enzyme involved in the cleavage of the alpha form of mannose. Its systematic name is alpha-D- ... Humans express the following three alpha-mannosidase isozymes: It can be utilized in experiments that determine the effects of ...

https://en.wikipedia.org/wiki/Alpha-Mannosidase

... may refer to: alpha-Mannosidase, an enzyme Mannosyl-oligosaccharide 1,2-alpha-mannosidase, an enzyme This ...

https://en.wikipedia.org/wiki/1,2-alpha-mannosidase

6-alpha-D-mannosidase (EC 3.2.1.163) is an enzyme that catalyzes the chemical reaction of separating the 1,6-linked alpha-D- ... The systematic name of this enzyme class is 1,6-alpha-mannosyl alpha-D-mannohydrolase. Athanasopoulos VI, Niranjan K, Rastall ... purification and characterisation of two novel alpha-D-mannosidases from Aspergillus phoenicis". Carbohydr. Res. 340 (4): 609- ... mannose residues in alpha-D-Manp-(1->6)-D-Manp. This enzyme belongs to the family of hydrolases, specifically those ...

https://en.wikipedia.org/wiki/1,6-alpha-D-mannosidase

... (EC 3.2.1.113, mannosidase 1A, mannosidase 1B, 1,2-alpha-mannosidase, exo-alpha- ... mannose-9 processing alpha-mannosidase, glycoprotein processing mannosidase I, mannosidase I, Man9-mannosidase, ManI, 1,2-alpha ... oligosaccharide alpha-D-mannohydrolase) is an enzyme with systematic name 2-alpha-mannosyl-oligosaccharide alpha-D- ... Mannosyl-oligosaccharide+1,2-alpha-mannosidase at the US National Library of Medicine Medical Subject Headings (MeSH) Portal: ...

https://en.wikipedia.org/wiki/Mannosyl-oligosaccharide_1,2-alpha-mannosidase

... (EC 3.2.1.130, glucosylmannosidase, endo-alpha-D-mannosidase, endo-alpha-mannosidase, ... Glycoprotein+endo-alpha-1,2-mannosidase at the US National Library of Medicine Medical Subject Headings (MeSH) Portal: Biology ... MANEA Lubas WA, Spiro RG (March 1987). "Golgi endo-alpha-D-mannosidase from rat liver, a novel N-linked carbohydrate unit ... This enzyme catalyses the following chemical reaction Hydrolysis of the terminal alpha-D-glucosyl-(1,3)-D-mannosyl unit from ...

https://en.wikipedia.org/wiki/Glycoprotein_endo-alpha-1,2-mannosidase

... (EC 3.2.1.101, exo-1,6-beta-mannanase, endo-alpha-1->6-D-mannanase, endo-1,6-beta-mannanase, ... mannan endo-1,6-beta-mannosidase, 1,6-alpha-D-mannan mannanohydrolase) is an enzyme with systematic name 6-alpha-D-mannan ... Mannan+endo-1,6-alpha-mannosidase at the US National Library of Medicine Medical Subject Headings (MeSH) Portal: Biology (EC ... This enzyme catalyses the following chemical reaction Random hydrolysis of (1->6)-alpha-D-mannosidic linkages in unbranched (1 ...

https://en.wikipedia.org/wiki/Mannan_endo-1,6-alpha-mannosidase

6-alpha-mannosidase (EC 3.2.1.114, mannosidase II, exo-1,3-1,6-alpha-mannosidase, alpha-D-mannosidase II, alpha-mannosidase II ... mannosidase, Golgi alpha-mannosidase II, ManII, 1,3(1,6)-alpha-D-mannosidase, 1,3-(1,6-)mannosyl-oligosaccharide alpha-D- ... Tulsiani DR, Hubbard SC, Robbins PW, Touster O (Apr 1982). "alpha-D-Mannosidases of rat liver Golgi membranes. Mannosidase II ... van den Elsen, J.M., Kuntz, D.A., Rose, D.R., "Structure of Golgi alpha-mannosidase II: a target for inhibition of growth and ...

https://en.wikipedia.org/wiki/Mannosyl-oligosaccharide_1,3-1,6-alpha-mannosidase

... (EC 3.2.1.137, exo-1,2-1,6-alpha-mannosidase, 1,2-1,6-alpha-D-mannan D-mannohydrolase) is ... doi:10.1016/0304-4165(89)90069-x. Mannan+exo-1,2-1,6-alpha-mannosidase at the US National Library of Medicine Medical Subject ... This enzyme catalyses the following chemical reaction Hydrolysis of (1->2)-alpha-D- and (1->6)-alpha-D- linkages in yeast ... Takegawa K, Miki S, Jikibara T, Iwahara S (1989). "Purification and characterization of exo-α-D-mannosidase from a Cellulomonas ...

https://en.wikipedia.org/wiki/Mannan_exo-1,2-1,6-alpha-mannosidase

... alpha-mannosidase (EC 3.2.1.113). Alpha-mannosidase is involved in the maturation of Asn-linked oligosaccharides. The enzyme ... "Isolation and expression of murine and rabbit cDNAs encoding an alpha 1,2-mannosidase involved in the processing of asparagine- ... hydrolyses terminal 1,2-linked alpha-D-mannose residues in the oligo-mannose oligosaccharide man(9)(glcnac)(2) in a calcium- ...

https://en.wikipedia.org/wiki/Glycoside_hydrolase_family_47

Glycoprotein endo-alpha-1,2-mannosidase is an enzyme that in humans is encoded by the MANEA gene. GRCh38: Ensembl release 89: ... "Entrez Gene: MANEA mannosidase, endo-alpha". Land A, Braakman I (2001). "Folding of the human immunodeficiency virus type 1 ... Spiro MJ, Bhoyroo VD, Spiro RG (1997). "Molecular cloning and expression of rat liver endo-alpha-mannosidase, an N-linked ...

https://en.wikipedia.org/wiki/MANEA

Mannan 1,2-(1,3)-alpha-mannosidase (EC 3.2.1.77, exo-1,2-1,3-alpha-mannosidase, 1,2-1,3-alpha-D-mannan mannohydrolase) is an ... Mannan+1,2-(1,3)-alpha-mannosidase at the US National Library of Medicine Medical Subject Headings (MeSH) Portal: Biology (EC ... Mode of action of the Arthrobacter alpha-mannosidase on yeast mannan". The Journal of Biological Chemistry. 244 (3): 1052-9. ... I. Purification and some properties of an alpha-mannosidase from an arthrobacter species". The Journal of Biological Chemistry ...

https://en.wikipedia.org/wiki/Mannan_1,2-(1,3)-a-mannosidase

Stegelmeier BL, Ralphs MH, Gardner DR, Molyneux RJ, James LF (October 1994). "Serum alpha-mannosidase activity and the ... As the estimated intake of swainsonine increased, blood serum alpha-mannosidase activity and albumin decreased, and alkaline ... Swainsonine inhibits a lysosomal enzyme, alpha-mannosidase. This results in abnormal accumulation of the molecules normally ... and analyzing blood serum for alpha-mannosidase activity and swainsonine. In mule deer, clinical signs of locoism are similar ...

https://en.wikipedia.org/wiki/Locoweed

... alpha-mannosidase (EC 3.2.1.24) (EC 3.2.1.114). Lysosomal alpha-mannosidase is necessary for the catabolism of N-linked ... non-reducing alpha-D-mannose residues in alpha-D-mannosides, and can cleave all known types of alpha-mannosidic linkages. ... The domain is predominantly found in the enzyme alpha-mannosidase. Henrissat B, Callebaut I, Fabrega S, Lehn P, Mornon JP, ... "The structure of bovine lysosomal alpha-mannosidase suggests a novel mechanism for low-pH activation". Journal of Molecular ...

https://en.wikipedia.org/wiki/Glycoside_hydrolase_family_38

Epididymis-specific alpha-mannosidase precursor Mannosidase alpha class 2B member core-specific lysosomal alpha-1,6-Mannosidase ... epididymis-specific alpha-mannosidase mannosidase, alpha, class 2B, member GRCh38: Ensembl release 89: ENSG00000013288 - ... "Entrez Gene: MAN2B2 mannosidase, alpha, class 2B, member 2". Land A, Braakman I (2001). "Folding of the human immunodeficiency ... Epididymis-specific alpha-mannosidase is an enzyme that in humans is encoded by the MAN2B2 gene. ...

https://en.wikipedia.org/wiki/MAN2B2

"Entrez Gene: MAN1B1 mannosidase, alpha, class 1B, member 1". Land A, Braakman I (2001). "Folding of the human immunodeficiency ... Endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase is an enzyme that in humans is encoded by the MAN1B1 gene ... Tremblay LO, Herscovics A (Nov 1999). "Cloning and expression of a specific human alpha 1,2-mannosidase that trims Man9GlcNAc2 ... 2-mannosidase gene involved in N-glycan maturation". Glycobiology. 8 (6): 585-95. doi:10.1093/glycob/8.6.585. PMID 9592125. v t ...

https://en.wikipedia.org/wiki/MAN1B1

"Entrez Gene: MAN1A1 mannosidase, alpha, class 1A, member 1". Land A, Braakman I (2001). "Folding of the human immunodeficiency ... Mannosyl-oligosaccharide 1,2-alpha-mannosidase IA is an enzyme that in humans is encoded by the MAN1A1 gene. This gene encodes ... 1997). "Man9-mannosidase from pig liver is a type-II membrane protein that resides in the endoplasmic reticulum. cDNA cloning ... Bieberich E, Bause E (1995). "Man9-mannosidase from human kidney is expressed in COS cells as a Golgi-resident type II ...

https://en.wikipedia.org/wiki/MAN1A1

"Molecular cloning and expression of cDNAs encoding human alpha-mannosidase II and a previously unrecognized alpha-mannosidase ... Alpha-mannosidase 2 is an enzyme that in humans is encoded by the MAN2A1 gene. This gene encodes a protein which is a member of ... "Entrez Gene: MAN2A1 mannosidase, alpha, class 2A, member 1". Land A, Braakman I (Aug 2001). "Folding of the human ... Moremen KW, Touster O, Robbins PW (Sep 1991). "Novel purification of the catalytic domain of Golgi alpha-mannosidase II. ...

https://en.wikipedia.org/wiki/MAN2A1

"Entrez Gene: MAN1A2 mannosidase, alpha, class 1A, member 2". Land A, Braakman I (2001). "Folding of the human immunodeficiency ... Mannosyl-oligosaccharide 1,2-alpha-mannosidase IB is an enzyme that in humans is encoded by the MAN1A2 gene. GRCh38: Ensembl ... 2-mannosidase gene involved in N-glycan maturation". Glycobiology. 8 (6): 585-595. doi:10.1093/glycob/8.6.585. PMID 9592125. " ...

https://en.wikipedia.org/wiki/MAN1A2

... is an enzyme which hydrolyses mannose. There are two types: alpha-Mannosidase beta-Mannosidase A deficiency is ... Mannosidases at the US National Library of Medicine Medical Subject Headings (MeSH) Portal: Biology v t e (EC 3.2.1, All stub ... A family of mannosidases are also responsible for processing newly formed glycoproteins in the endoplasmic reticulum into ...

https://en.wikipedia.org/wiki/Mannosidase

alpha-Mannosidase at the US National Library of Medicine Medical Subject Headings (MeSH) Portal: Medicine v t e (Use American ... Velmanase alfa is a recombinant form of human alpha-mannosidase. Velmanase alfa was approved for medical use in the European ... Malm D, Nilssen Ø (July 2019) [October 2001]. "Alpha-Mannosidosis". In Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJ, ... Velmanase alfa, sold under the brand name Lamzede, is a medication for the treatment of alpha-mannosidosis. ...

https://en.wikipedia.org/wiki/Velmanase_alfa

ER degradation-enhancing alpha-mannosidase-like 1 is an enzyme that in humans is encoded by the EDEM1 gene. GRCh38: Ensembl ... "Entrez Gene: EDEM1 ER degradation enhancer, mannosidase alpha-like 1". Nagase T, Seki N, Ishikawa K, et al. (1997). "Prediction ... 2001). "A novel ER alpha-mannosidase-like protein accelerates ER-associated degradation". EMBO Rep. 2 (5): 415-22. doi:10.1093/ ...

https://en.wikipedia.org/wiki/EDEM1

ER degradation-enhancing alpha-mannosidase-like 3 is an enzyme that in humans is encoded by the EDEM3 gene. GRCh38: Ensembl ... "Entrez Gene: EDEM3 ER degradation enhancer, mannosidase alpha-like 3". Maruyama K, Sugano S (1994). "Oligo-capping: a simple ...

https://en.wikipedia.org/wiki/EDEM3

ER degradation-enhancing alpha-mannosidase-like 2 is an enzyme that in humans is encoded by the EDEM2 gene. GRCh38: Ensembl ... "Entrez Gene: EDEM2 ER degradation enhancer, mannosidase alpha-like 2". Andersson B, Wentland MA, Ricafrente JY, et al. (1996 ...

https://en.wikipedia.org/wiki/EDEM2

Hosokawa N, Wada I, Hasegawa K, Yorihuzi T, Tremblay LO, Herscovics A, Nagata K (2001). "A novel ER alpha-mannosidase-like ... Jacob CA, Bodmer D, Spirig U, Battig P, Marcil A, Dignard D, Bergeron JJ, Thomas DY, Aebi M (2001). "Htm1p, a mannosidase-like ... Lenter M, Vestweber D (Apr 1994). "The integrin chains beta 1 and alpha 6 associate with the chaperone calnexin prior to ... Yos-9 recognizes mannose residues exposed after α-mannosidase removal of an outer mannose of misfolded glycoproteins. Calnexin ...

https://en.wikipedia.org/wiki/Calnexin

Disruption of Golgi alpha-mannosidase II with swainsonine induces hybrid-type glycans. These glycans have a Man5GlcNAc2 core ... It is a potent inhibitor of Golgi alpha-mannosidase II, an immunomodulator, and a potential chemotherapy drug. As a toxin in ... Dorling PR, Huxtable CR, Colegate SM (November 1980). "Inhibition of lysosomal alpha-mannosidase by swainsonine, an ... The inhibitory effect of swainsonine on Golgi Mannosidase II (GMII) was proposed to be due to its ability to bind in the GMII ...

https://en.wikipedia.org/wiki/Swainsonine

"Alpha-mannosidase-II deficiency results in dyserythropoiesis and unveils an alternate pathway in oligosaccharide biosynthesis ...

https://en.wikipedia.org/wiki/Jamey_Marth

B. Acid alpha-mannosidase activity Diagnosis is confirmed by measuring residual alpha-mannosidase activity in leukocytes or ... entry on Alpha-Mannosidosis OMIM entries on Alpha-Mannosidosis Alpha-mannosidosis type 1 at NIH's Office of Rare Diseases Alpha ... "Missense and nonsense mutations in the lysosomal alpha-mannosidase gene (MANB) in severe and mild forms of alpha-mannosidosis ... A defective alpha-mannosidase enzyme, which normally helps to break down complex sugars derived from glycoproteins in the ...

https://en.wikipedia.org/wiki/Alpha-mannosidosis

... shows no inhibitory action against mannosidase II or the endoplasmic reticulum alpha-mannosidase, and it weakly ... Kifunensine does not inhibit mannosidase II or the endoplasmic reticulum alpha-mannosidase. It weakly inhibits arylmannosidase ... Kifunensine inhibits human endoplasmic reticulum α-1,2-mannosidase I and Golgi Class I mannosidases IA, IB and IC with Ki ... Kifunensine is a potent inhibitor of the mannosidase I enzyme. It is 50 to 100 times more potent than deoxymannojirimycin - an ...

https://en.wikipedia.org/wiki/Kifunensine

Champion MJ, Brown JA, Shows TB (1979). "Assignment of cytoplasmic alpha-mannosidase (MANA) and confirmation of mitochondrial ... a partially unraveled alpha helix in the semi open form, and an alpha helix in the active form. The mitochondrial form of IDH2 ... Its main function is to catalyze the oxidative decarboxylation of isocitrate into alpha-ketoglutarate. Human isocitrate ... NADP+-dependent isocitrate dehydrogenase enhances the sensitivity of HeLa cells toward tumor necrosis factor-alpha and ...

https://en.wikipedia.org/wiki/IDH2

H is able to cleave each structure of this oligosaccharide as it is processed until the enzyme Golgi alpha-mannosidase II ...

https://en.wikipedia.org/wiki/Endoglycosidase_H

In addition, B. longum can uniquely ferment galactomannan-rich natural gum using glucosaminidases and alpha-mannosidases that ...

https://en.wikipedia.org/wiki/Bifidobacterium_longum

... mannosidase alpha class 1A member 1 (6q22.31) MB21D1: encoding protein Mab-21 domain containing 1 MCDR1: macular dystrophy, ... alpha 2(6p21.3) CYP21A2: cytochrome P450, family 21, subfamily A, polypeptide 2 (6p21.33) DHX16: DEAH-box helicase 16 (6p21.33 ...

https://en.wikipedia.org/wiki/Chromosome_6

Acquired generalized lipodystrophy (Lawrence syndrome, Lawrence-Seip syndrome) Adiposis dolorosa (Dercum's disease) Alpha-1 ... B-mannosidase deficiency Carotenosis Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy ... alpha-N-acetylgalactosaminidase deficiency) Schinzel-Giedion syndrome Scleroatrophic syndrome of Huriez (Huriez syndrome, ... Familial alpha-lipoprotein deficiency (Tangier disease) Familial amyloid polyneuropathy Familial apoprotein CII deficiency ...

https://en.wikipedia.org/wiki/List_of_skin_conditions

... alpha/beta-galactosidase, β-glucuronidase, α-fucosidase, α-mannosidase, and trypsin. Important dual virulence factors found in ...

https://en.wikipedia.org/wiki/Pseudogymnoascus_destructans

This domain occurs within alpha-1,2-mannosidases, which remove alpha-1,2-linked mannose residues from Man(9)(GlcNAc)(2) by ... 6-mannosidase EC 3.2.1.-, α-mannosidase EC 3.2.1.24, α-1,2-mannosidase EC 3.2.1.-, α-1,3-mannosidase EC 3.2.1.- and α-1,4- ... Glycoside hydrolase family 92 includes enzymes with mannosyl-oligosaccharide α-1,2-mannosidase EC 3.2.1.113, mannosyl- ... mannosidase EC 3.2.1.- activities. It includes enzymes critical for the maturation of N-linked oligosaccharides and ER- ...

https://en.wikipedia.org/wiki/Glycoside_hydrolase_family_92

... which has one alpha helix. Being made of beta sheets allows the structures to be more stable during chemical reactions. Both of ... glucosidases and mannosidases". J. Am. Chem. Soc. 65 (7): 1369-1380. doi:10.1021/ja01247a029. Liu Y, Zhou SH, Cheng YR, Chi Z, ...

https://en.wikipedia.org/wiki/Inulinase

Processing of asparagine-linked oligosaccharides by one or more rat liver Golgi alpha-D-mannosidases dependent on the prior ... Alpha Omega Alpha Honor Medical Society de Verteuil, Anthony (2014). Edward Lanza Joseph and the Jews in Trinidad. Port of ... action of UDP-N-acetylglucosamine: alpha-D-mannoside beta 2-N-acetylglucosaminyltransferase I". J Biol Chem. 255 (10): 4894-902 ...

https://en.wikipedia.org/wiki/Harry_Schachter

... a nervous tissue staining technique Golgi alpha-mannosidase II, an enzyme Golgi cell, a type of interneuron found in the ...

https://en.wikipedia.org/wiki/Golgi

... and alpha-mannosidases, through GnT-I-dependent and -independent truncation pathways. Studies on insect cell lines and in vivo ... From these two subunits, isoenzymes such as Hex A (one alpha and one beta subunit), Hex B (two beta subunits) and Hex S (two ... Moreover, α1,3-fucosyltransferase (FUT11/12) and β1,2-xylosyltransferase as well as α-mannosidase II were also reported to play ... Human Hex isoenzymes are assembled with alpha and beta subunits encoded by the HEXA and HEXB genes, respectively. ...

https://en.wikipedia.org/wiki/Paucimannosylation

... endo-beta-mannosidase) is an enzyme. This enzyme catalyses the following chemical reaction: Hydrolysis of the alpha-D-mannosyl ... Mannosylglycoprotein+endo-beta-mannosidase at the US National Library of Medicine Medical Subject Headings (MeSH) Portal: ... Ishimizu T, Sasaki A, Okutani S, Maeda M, Yamagishi M, Hase S (September 2004). "Endo-beta-mannosidase, a plant enzyme acting ... N-acetyl-beta-D-glucosaminyl sequence of glycoprotein to alpha-D-mannosyl-(1->6)-D-mannose and N-acetyl-beta-D-glucosaminyl-(1 ...

https://en.wikipedia.org/wiki/Mannosylglycoprotein_endo-beta-mannosidase

Beta-mannosidase Alpha-mannosidosis "Mannosidosis, beta A, lysosomal , Genetic and Rare Diseases Information Center (GARD) - an ... "OMIM Entry - * 609489 - MANNOSIDASE, BETA A, LYSOSOMAL; MANBA". www.omim.org. Retrieved 9 May 2018. Johnson, William (2015). ... Beta-mannosidosis, also called lysosomal beta-mannosidase deficiency, is a disorder of oligosaccharide metabolism caused by ... Beta-mannosidase function is consistent with, it being a lysosomal enzyme catalyzing and thus involved in degradation route for ...

https://en.wikipedia.org/wiki/Beta-mannosidosis

Alpha-mannosidase 2 MASS1: monogenic, audiogenic seizure susceptibility 1 homolog (mouse) MCC: Colorectal mutant cancer protein ... Prolyl 4-hydroxylase subunit alpha-2 PCBD2: Pterin-4-alpha-carbinolamine dehydratase 2 PELO: Pelota homolog PGGT1B: encoding ... alpha interacting protein (synphilin) SPEF2: Sperm flagellar protein 2 SPINK5: serine protease inhibitor Kazal-type 5 (LEKTI) ... alpha-induced protein 8 TTC37: Tetratricopeptide repeat domain 37 UPF0488: encodes G protein-coupled receptor protein signaling ...

https://en.wikipedia.org/wiki/Chromosome_5

... alpha-mannosidase II or N-acetylglycosaminyltransferase II is defective in CDA II. The anemia associated with CDA type II can ...

https://en.wikipedia.org/wiki/Congenital_dyserythropoietic_anemia_type_II

... alpha- and beta-glucuronidase, alpha-glucosidase, alpha-mannosidase, pyrazinamidase, pyrrolidonyl arylamidase, and urease. This ... It utilizes acid phosphatase, alkaline phosphatase, alpha-galactosidase, catalase, Pyrazinamidase, and urease. It is commonly ...

https://en.wikipedia.org/wiki/Arthrobacter_alkaliphilus

... a nervous tissue staining technique The enzyme Golgi alpha-mannosidase II Golgi cells of the cerebellum Golgi I nerve cells ( ...

https://en.wikipedia.org/wiki/Camillo_Golgi

Evidence for an alpha', beta Mechanism". J. Am. Chem. Soc. 94 8608-. Reich, H. J., Peake, S. L. (1978). "Hypervalent ... Inhibitors of B-Mannosidases?". Helv. Chim. Acta. 85 1118-. Cope, A.C.; Mehta, A.S. (1963). "Mechanism of the Hofmann ...

https://en.wikipedia.org/wiki/Ei_mechanism

2-alpha-mannosidase, an enzyme Manes (disambiguation) Maniyy, a term for sexual fluids in Islamic jurisprudence All pages with ...

https://en.wikipedia.org/wiki/Mani

Diagnosis of alpha-mannosidosis This test is not useful for establishing carrier status for alpha-mannosidosis. ... Alpha-mannosidosis is an autosomal recessive lysosomal storage disorder caused by reduced or absent acid alpha-mannosidase ... Alpha-mannosidosis is an autosomal recessive lysosomal storage disorder caused by reduced or absent acid alpha-mannosidase ... Alpha-mannosidosis is an autosomal recessive lysosomal storage disorder caused by reduced or absent acid alpha-mannosidase ...

https://www.mayocliniclabs.com/test-catalog/Overview/62511

admin January 28, 2022 Alpha-Mannosidase At the doses tested, there was cleavage of RIPK1 and RIPK3 and an increase in the ... admin July 7, 2021 Alpha-Mannosidase Many kits have already been formulated and optimized to be able to isolate EVs from serum ... admin November 9, 2021 Alpha-Mannosidase Here, we set out to determine the nature of the interaction between the sigma-1 ... admin February 19, 2022 Alpha-Mannosidase the increased loss of patients assessable for the selected biomarker as well as the ...

http://www.bioinf.org/category/alpha-mannosidase/

Alpha-Mannosidase B Deficiency. Here are the latest news stories from Alex TLC and about topics we think youll find useful. ...

https://www.alextlc.org/condition-known-as/alpha-mannosidase-b-deficiency/

... 2008 ...

https://www.genethon.com/publication/mannosidase-i-inhibition-rescues-the-human-alpha-sarcoglycan-r77c-recurrent-mutation/

Alpha-mannosidosis is a rare inherited disorder that causes problems in many organs and tissues of the body. Explore symptoms, ... Mutations in the MAN2B1 gene cause alpha-mannosidosis. This gene provides instructions for making the enzyme alpha-mannosidase ... Intracellular transport of human lysosomal alpha-mannosidase and alpha-mannosidosis-related mutants. Biochem J. 2004 Jul 15;381 ... Mutations in the MAN2B1 gene interfere with the ability of the alpha-mannosidase enzyme to perform its role in breaking down ...

https://medlineplus.gov/genetics/condition/alpha-mannosidosis

GOLGI ALPHA-MANNOSIDASE II complex with (2R,3R,4S)-2-({[(1R)-2-hydroxy-1-phenylethyl]amino}methyl)pyrrolidine-3,4-diol ... GOLGI ALPHA-MANNOSIDASE II complex with (2R,3R,4S)-2-({[(1R)-2-hydroxy-1-phenylethyl]amino}methyl)pyrrolidine-3,4-diol ... Englebienne, P. et al., Evaluation of docking programs for predicting binding of Golgi alpha-mannosidase II inhibitors: a ...

https://swissmodel.expasy.org/templates/2f18.1

Alpha-mannosidase catalyzes the degradation of accumulated mannose-containing oligosaccharides. Deficiency of alpha-mannosidase ... Alpha-Mannosidosis and Beta-Mannosidosis. Lysosomal alpha-mannosidase is a major exoglycosidase in the glycoprotein degradation ... A deficiency of this enzyme causes the lysosomal storage disease alpha-mannosidosis. Lysosomal alpha-D-mannosidase is involved ... Recent progress in lysosomal alpha-mannosidase and its deficiency. Exp Mol Med. 2001 Mar 31. 33(1):1-7. [QxMD MEDLINE Link]. ...

https://emedicine.medscape.com/article/1182830-overview

GOLGI ALPHA-MANNOSIDASE II IN COMPLEX WITH (2S,3R)-2-(HYDROXYMETHYL)- 1,2,3,6-TETRAHYDRO-3-PYRIDINOL - 6RRY , canSARS ... ALPHA-MAN-IIA, GMII, CG18802. TRICHOPLUSIA NI. FRUIT FLY. GOLGI ALPHA-MANNOSIDASE II,MAN II,GOLGI ALPHA-MANN ... ... GOLGI ALPHA-MANNOSIDASE II IN COMPLEX WITH (2S,3R)-2-(HYDROXYMETHYL)- 1,2,3,6-TETRAHYDRO-3-PYRIDINOL ... GOLGI ALPHA-MANNOSIDASE II IN COMPLEX WITH (2S,3R)-2-(HYDROXYMETHYL)- 1,2,3,6-TETRAHYDRO-3-PYRIDINOL ...

https://corona-1.cansar.icr.ac.uk/structure/6RRY/3d/6RRY_A

Compound: putative golgi alpha-mannosidase II. Species: Drosophila melanogaster [TaxId:7227]. Database cross-references and ... Description: Golgi alpha-mannosidase II complex with an amino-salacinol carboxylate analog. Class: hydrolase. Keywords: ...

http://scop.berkeley.edu/pdb/code=2fyv

... mannosidase-like (EDEM1) was proposed to extract terminally misfolded proteins from the calnexin folding cycle and target them ... Hosokawa, N.; Wada, I.; Hasegawa, K.; Yorihuzi, T.; Tremblay, L.O.; Herscovics, A.; Nagata, K. A novel ER alpha-mannosidase- ... Mannose processing proteins such as ER mannosidase I and EDEM (ER-degradation enhancing α-mannosidase-like) family of proteins ... Its structural homology with the ER mannosidase has led to the hypothesis that the mannosidase-like domain recognises high- ...

https://www.mdpi.com/1422-0067/21/10/3468

mannosidase 1, alpha (Man1a), Man1a1, PCR1 Protein level used designations for MAN1A. *alpha-1,2-mannosidase IA ... Progressively trim alpha-1,2-linked mannose residues from Man(9)GlcNAc(2) to produce Man(5)GlcNAc(2). ... mannosidase alpha class 1A member 1. *mannosyl-oligosaccharide 1,2-alpha-mannosidase IA ... mannosyl-oligosaccharide alpha-1,2-mannosidase. *processing alpha-1,2-mannosidase IA ...

https://www.antibodies-online.com/m/man1a-68667/

Isolation, characterization, and expression of cDNA encoding a rat liver endoplasmic reticulum alpha-mannosidase. J Biol Chem. ... Bischoff J, Brasel C. Regulation of P-selectin by tumor necrosis factor-alpha. Biochem Biophys Res Commun. 1995 May 05; 210(1): ...

https://connects.catalyst.harvard.edu/Profiles/display/Person/63431

alpha-mannosidosis: functional cloning of the lysosomal alpha-mannosidase cDNA and identification of a mutation in two affected ... Intracellular transport of human lysosomal alpha-mannosidase and alpha-mannosidosis-related mutants Biochemical Journal 2004 ... The Relationship between N-glycosylation of human lysosomal alpha-mannosidase and its intracellular transport 2003 ... Characterization of a novel alpha-mannosidosis-causing mutation and its use in leukocyte genotyping after bone marrow ...

https://uit.no/ansatte/person?p_document_id=41996

human alpha 1, 2-mannosidase. * 9) (IL) -1beta. * 10) D. melanogaster Surf-3 / rpL7a ...

https://bmcbioinformatics.biomedcentral.com/articles/10.1186/1471-2105-6-S1-S3

ER degradation enhancing alpha-mannosidase like protein 2. ISO. ClinVar Annotator: match by term: Maturity onset diabetes ... hepatocyte nuclear factor 4, alpha. ISO. ClinVar Annotator: match by term: Maturity onset diabetes mellitus in young. ClinVar. ... hepatocyte nuclear factor 4, alpha. ISO. ClinVar Annotator: match by term: Maturity-onset diabetes of the young type 3. ClinVar ... hepatocyte nuclear factor 4, alpha. ISO. ClinVar Annotator: match by term: FRTS4 WITH MODY , ClinVar Annotator: match by term: ...

https://rgd.mcw.edu/rgdweb/ontology/annot.html?species=Rat&acc_id=DOID:0050524

Alpha-mannosidase 2. Molecular Weight. 131139.485 Da. References. *Overington JP, Al-Lazikani B, Hopkins AL: How many drug ...

https://go.drugbank.com/drugs/DB03414

Mannosyl-oligosaccharide 1,2-alpha-mannosidase IA. External Links. *Kegg Reaction ID: R05982 ...

https://hmdb.ca/reactions/3561

mannosidase 2, alpha B1. Tssr78527. 8. 85083303 to 85083323 20. +. TSS region. transcription start site region 78527. ...

https://phenome.jax.org/markers/search?searchterm=8%3A84976678-85096734

Mutations in the alpha 1,2-mannosidase gene, MAN1B1, cause autosomal-recessive intellectual disability. Am J Hum Genet 89 (1), ...

https://www.molgen.mpg.de/publication-search/833442?person=%2Fpersons%2Fresource%2Fpersons50401

Contrary to a previous report, we were able to detect in vivo (but not in vitro) an extremely feeble ER alpha-mannosidase ... It has been postulated that creation of Man8GlcNAc2 isomer B (M8B) by endoplasmic reticulum (ER) alpha-mannosidase I ... Nevertheless, disruption of the alpha-mannosidase encoding gene almost totally prevented degradation of a misfolded ... This and other conflicting reports may be best explained by assuming that the role of ER mannosidase on glycoprotein ...

https://ri.conicet.gov.ar/handle/11336/42715

... through the accumulation of a covalent 2-deoxy-2-fluoro-alpha -D-mannosyl-beta -mannosidase 2A (2FMan-Man2A) enzyme ... Incubation of the beta -mannosidase Man2A from Cellulomonas fimi with 2-deoxy-2-fluoro-beta -D-mannosyl fluoride (2-FMan betaF ... This represents the first ever labelling and identification of an active-site nncleophile in a beta -mannosidase. ... Identification of Glu-519 as the catalytic nucleophile in beta-mannosidase 2A from Cellulomonas fimi. ...

https://www.chem.ubc.ca/identification-glu-519-catalytic-nucleophile-beta-mannosidase-2a-cellulomonas-fimi

... and beta-mannosidases. Although alkaloid 1 is known as a powerful inhibitor of lysosomal alpha-mannosidase (IC(50) = 0.02 ... was also a potent inhibitor of alpha-mannosidase with an IC(50) value of 4.6 microM. ... and alkaloid 5 was a moderate inhibitor of alpha- ...

https://erowid.org/references/refs_view.php?ID=7682&S=Gu_H

The Saccharomyces cerevisiae processing alpha 1,2-mannosidase is localized in the endoplasmic reticulum, independently of known ... Induction and substrate specificity of the lanosterol 14 alpha-demethylase from Saccharomyces cerevisiae Y222 Academic Article ...

https://experts.mcmaster.ca/display/vocab-mesh-saccharomyces-cerevisiae

Lysosomal alpha-mannosidase. $5.00. USD Add to cart. * Lysosomal protective protein. $5.00. USD Add to cart ... Alpha-synuclein. $5.00. USD Add to cart. * Aminoacyl tRNA synthase complex-interacting multifunctional protein 1. $5.00. USD ... Alpha-hemoglobin-stabilizing protein. $5.00. USD Add to cart. * ... Alpha-actinin-1. $5.00. USD Add to cart. * Alpha-amylase 1. $ ... Alpha-2-macroglobulin-P. $5.00. USD Add to cart. * ... Alpha-1-acid glycoprotein 1. $5.00. USD Add to cart. * Alpha-1- ...

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alpha 1-2.3.6 Mannosidase. (New England Biolabs). 80 units Zaloguj się Dodaj do koszyka ...

https://www.labjot.com/kategoria-produktu/analiza-bialek/enzymy-analiza-bialek/

Endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase, MA1B1_HUMAN Icon Locations in the PathwayBrowser Expand ...

https://reactome.org/content/detail/R-HSA-4960924

Although alkaloid 1 is known as a powerful inhibitor of lysosomal alpha-mannosidase (IC(50) = 0.02 microM), alkaloid 2, which has been thought to be an intermediate in the biosynthesis of 1, was also a potent inhibitor of alpha-mannosidase with an IC(50) value of 4.6 microM. (erowid.org)

Alpha-mannosidosis is an autosomal recessive lysosomal storage disorder caused by reduced or absent acid alpha-mannosidase enzyme activity. (mayocliniclabs.com)
This gene provides instructions for making the enzyme alpha-mannosidase. (medlineplus.gov)
Mutations in the MAN2B1 gene interfere with the ability of the alpha-mannosidase enzyme to perform its role in breaking down mannose-containing oligosaccharides. (medlineplus.gov)
An enzyme that catalyzes the HYDROLYSIS of terminal, non-reducing alpha-D-mannose residues in alpha-D-mannosides. (bvsalud.org)
Defects in the lysosomal form of the enzyme results in a buildup of mannoside intermediate metabolites and the disease ALPHA-MANNOSIDOSIS. (bvsalud.org)

In particular, alpha-mannosidase helps break down oligosaccharides containing a sugar molecule called mannose. (medlineplus.gov)

Kifunensin and 1-deoxymannojirimycin, being mannose homologues, would behave as inhibitors of the ER mannosidase or/and Htm1p/Mnl1p/EDEM putative lectin properties. (conicet.gov.ar)

ER-degradation enhancing α-mannosidase-like (EDEM1) was proposed to extract terminally misfolded proteins from the calnexin folding cycle and target them for degradation by ERAD. (mdpi.com)
It has been postulated that creation of Man8GlcNAc2 isomer B (M8B) by endoplasmic reticulum (ER) alpha-mannosidase I constitutes a signal for driving irreparably misfolded glycoproteins to proteasomal degradation. (conicet.gov.ar)
Nevertheless, disruption of the alpha-mannosidase encoding gene almost totally prevented degradation of a misfolded glycoprotein. (conicet.gov.ar)
This and other conflicting reports may be best explained by assuming that the role of ER mannosidase on glycoprotein degradation is independent of its enzymatic activity. (conicet.gov.ar)

G protein subunit alpha tr. (gsea-msigdb.org)

Evaluation of docking programs for predicting binding of Golgi alpha-mannosidase II inhibitors: a comparison with crystallography. (expasy.org)

Mutations in the MAN2B1 gene cause alpha-mannosidosis. (medlineplus.gov)

Determining enzymatic activity is the next step of the diagnostic workup for an individual clinically suspicious for an oligosaccharidosis with a positive screening result suggestive of alpha-mannosidosis. (mayocliniclabs.com)

Contrary to a previous report, we were able to detect in vivo (but not in vitro) an extremely feeble ER alpha-mannosidase activity in Schizosaccharomyces pombe. (conicet.gov.ar)
Alkaloids 3, 4, and 6 showed a potent inhibitory activity toward rat lysosomal beta-glucosidase, with IC(50) values of 2.1, 0.75, and 0.84 microM, respectively, and alkaloid 5 was a moderate inhibitor of alpha- and beta-mannosidases. (erowid.org)
It is most likely to have some alpha 1, 2-mannosidase activity. (biovisi.com)

This test is not useful for establishing carrier status for alpha-mannosidosis. (mayocliniclabs.com)
Alpha-mannosidosis is a rare inherited disorder that causes problems in many organs and tissues of the body. (medlineplus.gov)
The signs and symptoms of alpha-mannosidosis can range from mild to severe. (medlineplus.gov)
Individuals with this early-onset form of alpha-mannosidosis often do not survive past childhood. (medlineplus.gov)
Other individuals with alpha-mannosidosis experience milder signs and symptoms that appear later and progress more slowly. (medlineplus.gov)
People with later-onset alpha-mannosidosis may survive into their fifties. (medlineplus.gov)
Alpha-mannosidosis is estimated to occur in approximately 1 in 500,000 people worldwide. (medlineplus.gov)
Tissues and organs are damaged by the abnormal accumulation of oligosaccharides and the resulting cell death, leading to the characteristic features of alpha-mannosidosis. (medlineplus.gov)
Adult alpha-mannosidosis: clinical progression in the absence of demyelination. (medlineplus.gov)

Progressively trim alpha-1,2-linked mannose residues from Man(9)GlcNAc(2) to produce Man(5)GlcNAc(2). (antibodies-online.com)

This represents the first ever labelling and identification of an active-site nncleophile in a beta -mannosidase. (ubc.ca)
Here we report the cloning, characterization, and three-dimensional structure of the Cellvibrio mixtus GH5 beta-mannosidase (CmMan5A). (rcsb.org)
The 1.5 A crystal structure reveals that CmMan5A adopts a (beta/alpha)(8) barrel fold, and superimposition with GH5 endo-mannanases shows that dramatic differences in the length of three loops modify the active center accessibility and thus modulate the specificity from endo to exo. (rcsb.org)

Selective transport of alpha-mannosidase by autophagic pathways: structural basis for cargo recognition by ATG19 and ATG34. (hokudai.ac.jp)

Power of performed test with alpha = 0.050: 0.839). (healthweblognews.info)

alpha kinase 2 [Source:HGN. (gsea-msigdb.org)

Pour plus d'information, et notamment pour consulter la liste des tiers intervenant sur notre site, consultez la Politique de cookies accessible en bas de page. (genethon.com)

Lysosomal alpha-mannosidase with acidic pH optimum is ubiquitous in human tissues where is expressed in two major forms, A and B that are the product of a single gene located on chromosome 19. (nih.gov)
Mutations in the gene encoding for alpha-mannosidase cause alpha- mannosidosis, an autosomal recessive disease, resulting in the accumulation of unprocessed mannose containing oligosaccharide material. (nih.gov)
Mutations in the MAN2B1 gene cause alpha-mannosidosis. (medlineplus.gov)
This gene provides instructions for making the enzyme alpha-mannosidase. (medlineplus.gov)
Mutations in the MAN2B1 gene interfere with the ability of the alpha-mannosidase enzyme to perform its role in breaking down mannose-containing oligosaccharides. (medlineplus.gov)
Mutations in the alpha 1,2-mannosidase gene, MAN1B1, cause autosomal-recessive intellectual disability. (mpg.de)
10. Bi-allelic variants in the ER quality-control mannosidase gene EDEM3 cause a congenital disorder of glycosylation. (nih.gov)
This gene encodes a plasma alpha-L-fucosidase, which represents 10-20% of the total cellular fucosidase activity. (nih.gov)
C3101 Genetic Disorder C99147 Neonatal Research Network Terminology C98699 5 Alpha Steroid Reductase 2 Deficiency 3-Oxo-5 Alpha-Steroid Delta 4-Dehydrogenase Deficiency An autosomal recessive inherited disorder caused by mutations in the SRD5A2 gene. (nih.gov)

It is characterized by deficiency of the enzyme steroid 5-alpha reductase 2 that catalyzes the conversion of testosterone to dihydrotestosterone. (nih.gov)
Alpha-mannosidosis is lysosomal storage disorder caused by deficiency of lysosomal alpha mannosidase ( MAN2B1 ). (jupiter.no)

LAMAN: lysosomal acid alpha-mannosidase for alpha-mannosidosis. (m6ptherapeutics.com)
Recombinant AAV serotype 6 (rAAV6) is used to deliver the human lysosomal alpha-mannosidase (LAMAN) (MAN2B) cDNA sequence to the choroid plexus epithelia. (nih.gov)

The serum alpha fetoprotein (AFP), beta individual chorionic gonadotrophic hormone ( HCG) and lactate dehydrogenase (LDH) beliefs had been 16,093 ng/mL, 1.2 Miu/mL and 1013 U/L, respectively. (columbiagypsy.net)
Furthermore, we identified the current presence of a mannosidase in mouse serum that transformed most Guy8/9 to Guy6 after 24 h. was chosen for this function, and optimized response circumstances had been discovered that generated homogeneous Guy5 glycoform nearly. (bassresearch.com)

Hydrolysis of terminal, non-reducing alpha-D-mannose residues in alpha-D-mannosides. (uniprot.org)
Glycoside hydrolases that catalyze the hydrolysis of alpha or beta linked MANNOSE . (bvsalud.org)

Progressively trim alpha-1,2-linked mannose residues from Man 9 GlcNAc 2 to produce Man 5 GlcNAc 2 . (uniprot.org)
Trim alpha-1,2-linked mannose residues from Man(9)GlcNAc(2) to produce first Man(8)GlcNAc(2) then Man(6)GlcNAc and a small amount of Man(5)GlcNAc. (nih.gov)
2)-linked alpha-D-mannose residues in the oligo-mannose oligosaccharide Man(9)(GlcNAc)(2). (systemsbiology.net)
Kifunensine, a potent and selective inhibitor of class I α-mannosidases isolated from Actinomycete, prevents α-mannosidases I from trimming mannose residues on glycoproteins. (medchemexpress.com)

Furthermore, no fucosylated Man9GlcNAc2 glycans were detected following inhibition of alpha-mannosidase I with kifunensine. (ox.ac.uk)
Kifunensine, an alkaloid from actinomycete Kitasatosporia kifunense 9482, is the most efficient nhibitor of a-mannosidase I, blocking N-glycan synthesis at an8GlcNAc 2 (Man8) or Man9GlcNAc 2 (Man9) stage [3] . (medchemexpress.com)

Alpha-L-fucosidase is responsible for hydrolyzing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins. (nih.gov)

Purification and characterization of UDP-N-acetylglucosamine: alpha-6-D-mannoside beta 1-6N-acetylglucosaminyltransferase (N-acetylglucosaminyltransferase V) from a human lung cancer cell line. (semanticscholar.org)
D-mannoside .beta.2-N-acetylglucosaminyltransferase I. Separation from UDP-N-acetylglucosamine: .alpha. (genome.jp)
Defects in the lysosomal form of the enzyme results in a buildup of mannoside intermediate metabolites and the disease ALPHA-MANNOSIDOSIS . (nih.gov)

Structural basis for catalysis and inhibition of N-glycan processing class I alpha 1,2-mannosidases. (expasy.org)

Alpha-mannosidosis is a rare inherited disorder that causes problems in many organs and tissues of the body. (medlineplus.gov)
The signs and symptoms of alpha-mannosidosis can range from mild to severe. (medlineplus.gov)
Individuals with this early-onset form of alpha-mannosidosis often do not survive past childhood. (medlineplus.gov)
Other individuals with alpha-mannosidosis experience milder signs and symptoms that appear later and progress more slowly. (medlineplus.gov)
People with later-onset alpha-mannosidosis may survive into their fifties. (medlineplus.gov)
Alpha-mannosidosis is estimated to occur in approximately 1 in 500,000 people worldwide. (medlineplus.gov)
Tissues and organs are damaged by the abnormal accumulation of oligosaccharides and the resulting cell death, leading to the characteristic features of alpha-mannosidosis. (medlineplus.gov)
Adult alpha-mannosidosis: clinical progression in the absence of demyelination. (medlineplus.gov)
LAMZEDE is recombinant human lysosomal alpha-mannosidase indicated for the treatment of non-central nervous system manifestations of alpha-mannosidosis in adult and pediatric patients. (nih.gov)
Based on a scientific breakthrough by researchers Stuart Kornfeld and Lin Liu in 2017, we successfully created a technology platform to regulate the M6P levels on lysosomal enzymes, allowing us to potentially treat even LSDs where the required enzymes have naturally low M6P levels, such as mucopolysaccaridosis IIIB (MPS IIIB) and alpha-mannosidosis. (m6ptherapeutics.com)
Performing regular review of the literature on alpha-Mannosidosis mutations and their functional impact. (jupiter.no)
Performing research on the spectrum of alpha-Mannosidosis mutations, as well as on the impact of mutations on enzyme activity, structure, processing, localization and clinical features. (jupiter.no)
Alpha-mannosidosis is inherited in an autosomal recessive manner . (jupiter.no)
The growth is mainly due to increasing prevalence of alpha mannosidosis, use of orphan drugs, fee reductions and tax credits, increasing investment in the rare disease treatment and advancements in treatment drugs. (siliconvalleylivenews.com)
The increasing investments on research are expected to contribute to the growth of the alpha mannosidosis market in the forecast period. (siliconvalleylivenews.com)
The alpha mannosidosis market consists of sales of therapies for alpha mannosidosis (organizations, sole traders and partnerships) that provides treatment for alpha mannosidosis, which is a lysosomal storage disorder. (siliconvalleylivenews.com)
Alpha-mannosidosis is a rare genetic inherited condition that affects the organs and tissues of the person suffering from the disease. (siliconvalleylivenews.com)
The advancements in treatment for alpha mannosidosis like pharmacological therapy are gaining traction in the alpha mannosidosis market. (siliconvalleylivenews.com)
The global alpha mannosidosis market is further segmented based on therapy type, indication, end-user and geography. (siliconvalleylivenews.com)
By Geography: The global alpha mannosidosis market is segmented into North America, South America, Asia-Pacific, Eastern Europe, Western Europe, Middle East and Africa. (siliconvalleylivenews.com)
Among these regions, the North American alpha mannosidosis market accounts for the largest share in the global alpha mannosidosis market. (siliconvalleylivenews.com)
Alpha Mannosidosis Global Market Report 2021 is one of a series of new reports from The Business Research Company that provides alpha mannosidosis global market overviews, analyzes and forecasts market size and growth for the global alpha mannosidosis global market, alpha mannosidosis global market share, alpha mannosidosis global market players, alpha mannosidosis global market segments and geographies, alpha mannosidosis global market's leading competitors' revenues, profiles and market shares. (siliconvalleylivenews.com)
The alpha mannosidosis global market report identifies top countries and segments for opportunities and strategies based on market trends and leading competitors' approaches. (siliconvalleylivenews.com)
Alpha Mannosidosis Market Organizations Covered: Chiesi Farmaceutici S.p.A.., Nuo Therapeutics, and Zymenex. (siliconvalleylivenews.com)

Biochemical analysis using bacterially produced recombinant AtMANI isoforms revealed that both AtMANI isoforms encode 1-deoxymannojirimycin-sensitive -mannosidase I and act on Man 8 GlcNAc 2 and Man 9 GlcNAc 2 structures to yield Man 5 GlcNAc 2 . (scialert.net)
There are multiple isoforms of alpha-mannosidase, each having its own specific cellular location and pH optimum. (nih.gov)

6. In vitro mannosidase activity of EDEM3 against asparagine-linked oligomannose-type glycans. (nih.gov)

14. Mannosidase IA is in Quality Control Vesicles and Participates in Glycoprotein Targeting to ERAD. (nih.gov)
The removal of an alpha-1,2-linked mannose from the B-chain of a glycoprotein oligosaccharide in the endoplasmic reticulum. (cyverse.org)

mannosidase alpha class. (gsea-msigdb.org)
Calnexin also functions as a chaperone for the folding of MHC class I alpha chain in the membrane of the ER. (primidi.com)
Labetalolum belongs to a class of drugs called Beta-Blockers, Alpha Activity. (hypertension365.us)

18. EDEM1's mannosidase-like domain binds ERAD client proteins in a redox-sensitive manner and possesses catalytic activity. (nih.gov)

In particular, alpha-mannosidase helps break down oligosaccharides containing a sugar molecule called mannose. (medlineplus.gov)

serpin peptidase inhibitor, clade F (alpha. (wikigenes.org)

12. Trimming of glucosylated N-glycans by human ER α1,2-mannosidase I. (nih.gov)

CardioVascular Support proprietary blend: Alpha Lipoic Acid (as Thioctic Acid), Coenzyme Q`0, (CO Q 10)(as Ubidecarenone), Grape Seed Extract (95% Proanthocyanidins)(Vitis vinfera)(seed), Acai Berry 4:1 Extract (Euterpe oleracea)(fruit, Resveratrol 50% (from Japanese Knotweed) (Polygonum cuspidatum)(root), Garlic (Allum sativum)(bulb). (bodyandfitness.com)
Garlic (allium sativum) (bulb), alpha lipoic acid (as thioctic acid), grape seed extract (95% proanthocyanidins) (vitis vinifera) (seed), acai berry 4:1 extract (Euterpe oleracea) (fruit), coenzyme Q10 (CoQ10) (as ubidecarenone), resveratrol 50% (from japanese knotweed) (polygonum cuspidatum) (root). (bodyandfitness.com)

N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase (NAGPA), also known as uncovering enzyme, removes the covering GlcNAc group, thereby exposing mannose 6-phosphate. (m6ptherapeutics.com)

GAA: acid alpha-glucosidase for Pompe disease. (m6ptherapeutics.com)

Members of this family are alpha-mannosidases that catalyse the. (systemsbiology.net)

Primary tests with commercially obtainable mannosidase revealed the fact that addition of calcium mineral as well as the expansion of response time seemed to improve the LY 2874455 trimming response, which yielded higher Guy5 articles. (bassresearch.com)

collagen type XXI alpha 1 chain [Sou. (gsea-msigdb.org)

2f18.1 | SWISS-MODEL Template Library (swissmodel.expasy.org)

Alpha-mannosidosis: MedlinePlus Genetics (medlineplus.gov)

MAN2B1 gene: MedlinePlus Genetics (medlineplus.gov)

Profile | Biosciences | University of Exeter (biosciences.exeter.ac.uk)

Anatomy16

Organisms13

Diseases2

Chemicals and Drugs124

Analytical, Diagnostic and Therapeutic Techniques and Equipment22

Phenomena and Processes40

Disciplines and Occupations1

Technology, Industry, Agriculture1

Information Science4

Endometrial lysosomal enzyme activity in normal cycling endometrium. (1/346)

Mid2 is a putative sensor for cell integrity signaling in Saccharomyces cerevisiae. (2/346)

Purification and biochemical characterization of two soluble alpha-mannosidases from Candida albicans. (3/346)

Characterization of a soluble class I alpha-mannosidase in human serum. (4/346)

Androgen regulation of glycosidase secretion in epithelial cell cultures from human epididymis. (5/346)

Topological and functional characterization of the N-glycans of soybean (Glycine max) agglutinin. (6/346)

Targeted disruption of the lysosomal alpha-mannosidase gene results in mice resembling a mild form of human alpha-mannosidosis. (7/346)

Purification and properties of major alpha-D-mannosidase in the luminal fluid of porcine epididymis. (8/346)

Mannosidases

alpha-Mannosidase

Swainsonine

1-Deoxynojirimycin

Oligosaccharides

Mannose

Golgi Apparatus

Polysaccharides

Alkaloids

Mannosidase Deficiency Diseases

alpha 1-Antitrypsin

Imino Furanoses

Endoplasmic Reticulum

Carbohydrate Sequence

Indolizines

Mannosyl-Glycoprotein Endo-beta-N-Acetylglucosaminidase

Glycosylation

Glycoproteins

Glucosamine

Molecular Sequence Data

Carbohydrate Conformation

Receptors, Adrenergic, alpha

Pichia

Hypoxia-Inducible Factor 1, alpha Subunit

Asparagine

Substrate Specificity

Brefeldin A

Kinetics

Base Sequence

alpha-Glucosidases

Hexosaminidases

Glycoside Hydrolases

Protein Processing, Post-Translational

Amino Acid Sequence

N-Acetylglucosaminyltransferases

Acetylglucosaminidase

Monensin

alpha7 Nicotinic Acetylcholine Receptor

Immunosorbent Techniques

p-Chloromercuribenzoic Acid

Recombinant Proteins

Glucosidases

Cloning, Molecular

Liver

Integrin alpha3beta1

Integrin alpha4

Integrin alpha6

Integrin alpha5beta1

Cricetinae

Cell Line

DNA, Complementary

Acetylglucosamine

Intracellular Membranes

Chromatography

Integrin alpha4beta1

Coat Protein Complex I

Interleukin-1alpha

Transfection

Integrin alpha2beta1

Galactosyltransferases

Protein Binding

Receptors, Adrenergic, alpha-1

Integrin alpha5

Integrin alpha1beta1

Mannans

Membrane Proteins

Receptors, Adrenergic, alpha-2

Sequence Homology, Amino Acid

Tunicamycin

Integrin alpha6beta1

Binding Sites

Hydrolysis

Cell Compartmentation

Enzyme Inhibitors

CHO Cells

Integrin alpha6beta4

Integrin alpha Chains

Microsomes, Liver

Integrins

Integrin alpha1