Steroidal compounds related to PROGESTERONE, the major mammalian progestational hormone. Progesterone congeners include important progesterone precursors in the biosynthetic pathways, metabolites, derivatives, and synthetic steroids with progestational activities.
A heterogeneous family of water-soluble structural proteins found in cells of the vertebrate lens. The presence of these proteins accounts for the transparency of the lens. The family is composed of four major groups, alpha, beta, gamma, and delta, and several minor groups, which are classed on the basis of size, charge, immunological properties, and vertebrate source. Alpha, beta, and delta crystallins occur in avian and reptilian lenses, while alpha, beta, and gamma crystallins occur in all other lenses.
The basic subunit of beta-crystallins.
The acidic subunit of beta-crystallins.
A transparent, biconvex structure of the EYE, enclosed in a capsule and situated behind the IRIS and in front of the vitreous humor (VITREOUS BODY). It is slightly overlapped at its margin by the ciliary processes. Adaptation by the CILIARY BODY is crucial for OCULAR ACCOMMODATION.
A subclass of crystallins that found in the lens (LENS, CRYSTALLINE) of VERTEBRATES. Gamma-crystallins are similar in structure to BETA-CRYSTALLINS in that they both form into a Greek key-like structure. They are composed of monomeric subunits.
A class of crystallins that provides refractive power and translucency to the lens (LENS, CRYSTALLINE) in VERTEBRATES. Beta-crystallins are similar in structure to GAMMA-CRYSTALLINS in that they both contain Greek key motifs. Beta-crystallins exist as oligomers formed from acidic (BETA-CRYSTALLIN A CHAIN) and basic (BETA-CRYSTALLIN B CHAIN) subunits.
A protein phytotoxin from the seeds of Ricinus communis, the castor oil plant. It agglutinates cells, is proteolytic, and causes lethal inflammation and hemorrhage if taken internally.
One of the subunits of alpha-crystallins. Unlike ALPHA-CRYSTALLIN B CHAIN the expression of ALPHA-CRYSTALLIN A CHAIN is limited primarily to the lens (LENS, CRYSTALLINE).
A subclass of crystallins that provides the majority of refractive power and translucency to the lens (LENS, CRYSTALLINE) in VERTEBRATES. Alpha-crystallins also act as molecular chaperones that bind to denatured proteins, keep them in solution and thereby maintain the translucency of the lens. The proteins exist as large oligomers that are formed from ALPHA-CRYSTALLIN A CHAIN and ALPHA-CRYSTALLIN B CHAIN subunits.
One of the alpha crystallin subunits. In addition to being expressed in the lens (LENS, CRYSTALLINE), alpha-crystallin B chain has been found in a variety of tissues such as HEART; BRAIN; MUSCLE; and KIDNEY. Accumulation of the protein in the brain is associated with NEURODEGENERATIVE DISEASES such as CREUTZFELDT-JAKOB SYNDROME and ALEXANDER DISEASE.
A subclass of crystallins found in the lens (LENS, CRYSTALLINE) in BIRDS and REPTILES. They are inactive forms of the enzyme argininosuccinate lyase.
Partial or complete opacity on or in the lens or capsule of one or both eyes, impairing vision or causing blindness. The many kinds of cataract are classified by their morphology (size, shape, location) or etiology (cause and time of occurrence). (Dorland, 27th ed)
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 order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
A toxic lectin from the seeds of jequirity, Abrus precatorius L. Very active poison. Five different proteins have so far been isolated: Abrus agglutinin, the component responsible for: hemagglutinating activity, & abrins a-d, the toxic principals each consisting of two peptide chains are held together by disulfide bonds.
A genus of poisonous snakes of the subfamily Elapinae of the family ELAPIDAE. They comprise the kraits. Twelve species are recognized and all inhabit southeast Asia. They are considered extremely dangerous. (Moore: Poisonous Snakes of the World, 1980, p120)
An interleukin-1 subtype that is synthesized as an inactive membrane-bound pro-protein. Proteolytic processing of the precursor form by CASPASE 1 results in release of the active form of interleukin-1beta from the membrane.
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.
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
An 11-kDa protein associated with the outer membrane of many cells including lymphocytes. It is the small subunit of the MHC class I molecule. Association with beta 2-microglobulin is generally required for the transport of class I heavy chains from the endoplasmic reticulum to the cell surface. Beta 2-microglobulin is present in small amounts in serum, csf, and urine of normal people, and to a much greater degree in the urine and plasma of patients with tubular proteinemia, renal failure, or kidney transplants.
Measurement of the index of refraction (the ratio of the velocity of light or other radiation in the first of two media to its velocity in the second as it passes from one into the other).
A family of cellular proteins that mediate the correct assembly or disassembly of polypeptides and their associated ligands. Although they take part in the assembly process, molecular chaperones are not components of the final structures.
Lists of persons or organizations, systematically arranged, usually in alphabetic or classed order, giving address, affiliations, etc., for individuals, and giving address, officers, functions, and similar data for organizations. (ALA Glossary of Library and Information Science, 1983)
Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).
The property of antibodies which enables them to react with some ANTIGENIC DETERMINANTS and not with others. Specificity is dependent on chemical composition, physical forces, and molecular structure at the binding site.
Antibodies produced by a single clone of cells.
The removal of a cataractous CRYSTALLINE LENS from the eye.
Genes that influence the PHENOTYPE both in the homozygous and the heterozygous state.
The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition.
The co-inheritance of two or more non-allelic GENES due to their being located more or less closely on the same CHROMOSOME.
That part of the genome that corresponds to the complete complement of EXONS of an organism or cell.
An enzyme formed from PROTHROMBIN that converts FIBRINOGEN to FIBRIN.
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)
DNA molecules capable of autonomous replication within a host cell and into which other DNA sequences can be inserted and thus amplified. Many are derived from PLASMIDS; BACTERIOPHAGES; or VIRUSES. They are used for transporting foreign genes into recipient cells. Genetic vectors possess a functional replicator site and contain GENETIC MARKERS to facilitate their selective recognition.
Exclusive legal rights or privileges applied to inventions, plants, etc.
Individuals enrolled in a school or formal educational program.
The teaching staff and members of the administrative staff having academic rank in a medical school.
The teaching staff and members of the administrative staff having academic rank in an educational institution.
Individuals enrolled in a school of medicine or a formal educational program in medicine.
Critical and exhaustive investigation or experimentation, having for its aim the discovery of new facts and their correct interpretation, the revision of accepted conclusions, theories, or laws in the light of newly discovered facts, or the practical application of such new or revised conclusions, theories, or laws. (Webster, 3d ed)
A biologic alkylating agent that exerts its cytotoxic effects by forming DNA ADDUCTS and DNA interstrand crosslinks, thereby inhibiting rapidly proliferating cells. The hydrochloride is an antineoplastic agent used to treat HODGKIN DISEASE and LYMPHOMA.
Copies of a work or document distributed to the public by sale, rental, lease, or lending. (From ALA Glossary of Library and Information Science, 1983, p181)
Glycosidic antibiotic from Streptomyces griseus used as a fluorescent stain of DNA and as an antineoplastic agent.
Cysts found in the jaws and arising from epithelium involved in tooth formation. They include follicular cysts (e.g., primordial cyst, dentigerous cyst, multilocular cyst), lateral periodontal cysts, and radicular cysts. They may become keratinized (odontogenic keratocysts). Follicular cysts may give rise to ameloblastomas and, in rare cases, undergo malignant transformation.
A region extending from the PONS & MEDULLA OBLONGATA through the MESENCEPHALON, characterized by a diversity of neurons of various sizes and shapes, arranged in different aggregations and enmeshed in a complicated fiber network.
Software used to locate data or information stored in machine-readable form locally or at a distance such as an INTERNET site.
Organized activities related to the storage, location, search, and retrieval of information.
Materials that have a limited and usually variable electrical conductivity. They are particularly useful for the production of solid-state electronic devices.

Genetic heterogeneity of the Coppock-like cataract: a mutation in CRYBB2 on chromosome 22q11.2. (1/70)

PURPOSE: To identify the genetic defect for the Coppock-like cataract (CCL) affecting a Swiss family, which defect was unlinked to the chromosome 2q33-35 CCL locus. METHODS: A large family was characterized for linkage analysis by slit lamp examination or by the review of drawings made before cataract extraction. The affection status was attributed before genotyping, and the genotyping was masked to the affection status. Two-point and multipoint linkage analyses were performed using the MLINK and the LINKMAP components of the LINKAGE program package (ver. 5.1), respectively. Mutational analysis of candidate genes was performed by a combination of direct cycle sequencing and an amplification refractory mutation system assay. RESULTS: Ten individuals were affected with the CCL phenotype. The disease was autosomal dominant and appeared to be fully penetrant. A new CCL locus was identified on chromosome 22q11.2 within a 11.67-cM interval (maximum lod score [Zmax] = 4.14; theta = 0). Mutational analysis of the CRYBB2 candidate gene identified a disease-causing mutation in exon 6. This sequence change was identical with that previously described to be associated with the cerulean cataract, a clinically distinct entity. CONCLUSIONS: The CCL phenotype is genetically heterogeneous with a second gene on chromosome 22q11.2, CRYBB2. The CCL and the cerulean cataract are two distinct clinical entities associated with the same genetic defect. This work provides evidence for a modifier factor that influences cataract formation and that remains to be identified.  (+info)

Expression of betaB(2)-crystallin mRNA and protein in retina, brain, and testis. (2/70)

PURPOSE: To evaluate the expression of betaB(2)-crystallin mRNA and protein in rat, bovine, and human nonlens and nonocular tissues. METHODS: betaB(2)-crystallin mRNA levels were detected by RT-PCR. betaB(2)-crystallin protein was purified from rat and bovine tissues by FPLC chromatography. FPLC fractions were analyzed by immunoblotting. The identity of betaB(2)-crystallin protein, isolated from the retina, was confirmed by protein microsequencing. RESULTS: betaB(2)-crystallin transcript was detected in rat brain, rat testis, and human retina by RT-PCR. betaB(2)-crystallin transcript was not found in rat lung, heart, ovary, spleen, thymus, kidney, and liver or in human brain and testis. betaB(2)-crystallin protein was partially purified from and its identity confirmed in rat brain, rat testis, and bovine retina. The bovine retinal protein was further confirmed to be authentic betaB(2)-crystallin by protein microsequencing. CONCLUSIONS: These results establish that betaB(2)-crystallin mRNA and protein are expressed in tissues outside of the lens and outside of the eye including retina, brain, and testis. Extralenticular and extraocular expression of betaB(2)-crystallin, coupled with its participation in phosphorylation pathways, suggests that it has nonrefractive functions in these tissues.  (+info)

Aey2, a new mutation in the betaB2-crystallin-encoding gene of the mouse. (3/70)

PURPOSE: During an ethylnitrosourea (ENU) mutagenesis screen, mice were tested for the occurrence of dominant cataracts. One particular mutant was found that caused progressive opacity and was referred to as Aey2. The purpose of the study was to provide a morphologic description, to map the mutant gene, and to characterize the underlying molecular lesion. METHODS: Isolated lenses were photographed, and histologic sections of the eye were analyzed according to standard procedures. Linkage analysis was performed using a set of microsatellite markers covering all autosomal chromosomes. cDNA from candidate genes was amplified after reverse transcription of lens mRNA. RESULTS: The cortical opacification visible at eye opening progressed to an anterior suture cataract and reached its final phenotype as total opacity at 8 weeks of age. There was no obvious difference between heterozygous and homozygous mutants. The mutation was mapped to chromosome 5 proximal to the marker D5Mit138 (8.7 +/- 4.2 centimorgan [cM]) and distal to D5Mit15 (12.8 +/- 5.4 cM). No recombinations were observed to the markers D5Mit10 and D5Mit25. This position makes the genes within the betaA4/betaB-crystallin gene cluster excellent candidate genes. Sequence analysis revealed a mutation of T-->A at position 553 in the Crybb2 gene, leading to an exchange of Val for GLU: It affects the same region of the Crybb2 gene as in the Philly mouse. Correspondingly, the loss of the fourth Greek key motif is to be expected. CONCLUSIONS: The Aey2 mutant represents the second allele of Crybb2 in mice. Because an increasing number of beta- and gamma-crystallin mutations have been reported, a detailed phenotype-genotype correlation will allow a clearer functional understanding of beta- and gamma-crystallins.  (+info)

Identification and properties of anti-chaperone-like peptides derived from oxidized bovine lens betaL-crystallins. (4/70)

Thermal aggregation of betaL-crystallin was higher in the presence of peptide fragments generated from oxidized and trypsin-digested betaL-crystallin compared with thermal aggregation of the control proteins without oxidized betaL-crystallin fragments. Increased aggregation of betaL-crystallin was also observed despite the presence of alpha-crystallin (which has anti-aggregating properties) in the system. Self-aggregation of the oxidized betaL-crystallin fragments per se was not observed under the experimental conditions. Reverse-phase HPLC analysis of the precipitate obtained after heating a mixture of betaL-crystallin and oxidized betaL-crystallin fragments revealed that more than one peptide co-precipitates with betaL-crystallin. Electrospray mass spectrometry analysis of the peptides revealed that the molecular weight(s) of the peptides ranged from 1400-1800. Tandem mass spectrometry and a data base search revealed that two of the peptides originated from betaA4-crystallin (LTIFEQENFLGR, residues 121-132) and betaB3-crystallin (AINGTWVGYEFPGYR, residues 153-167) respectively. Oxidized synthetic peptides representing the same sequence were also found to enhance the aggregation of betaL-crystallin in a manner similar to oxidized lens betaL-crystallin peptides. These data suggest that the polypeptides generated after oxidation and proteolysis of betaL-crystallins interact with denaturing proteins and facilitate their aggregation and light scattering, thus behaving like anti-chaperones.  (+info)

Decreased heat stability and increased chaperone requirement of modified human betaB1-crystallins. (5/70)

PURPOSE: To determine how deamidation and partial loss of the N- and C-terminal extensions alter the heat stability of betaB1-crystallin. METHODS: Human lens betaB1, a deamidated betaB1, Q204E, and alphaA-crystallins were expressed. Truncated betaB1 was generated by proteolytic removal of part of its terminal extensions. The aggregation and precipitation of these proteins due to heating was monitored by circular dichroism and light scattering. The effect of heat on the stability of both monomers and oligomers was investigated. The flexibility of the extensions in wild type and deamidated betaB1 was assessed by 1H NMR spectroscopy. RESULTS: With heat, deamidated betaB1 precipitated more readily than wild type betaB1. Similar effects were obtained for either monomers or oligomers. Flexibility of the N-terminal extension in deamidated betaB1 was significantly reduced compared to the wild type protein. Truncation of the extensions further increased the rate of heat-induced precipitation of deamidated betaB1. The presence of the molecular chaperone, alphaA-crystallin, prevented precipitation of modified betaB1s. More alphaA was needed to chaperone the truncated and deamidated betaB1 than deamidated betaB1 or truncated betaB1. CONCLUSIONS: Deamidation and truncation of betaB1 led to destabilization of the protein and decreased stability to heat. Decreased stability of lens crystallins may contribute to their insolubilization and cataract formation.  (+info)

A nonsense mutation in CRYBB1 associated with autosomal dominant cataract linked to human chromosome 22q. (6/70)

Autosomal dominant cataract is a clinically and genetically heterogeneous lens disorder that usually presents as a sight-threatening trait in childhood. Here we have mapped dominant pulverulent cataract to the beta-crystallin gene cluster on chromosome 22q11.2. Suggestive evidence of linkage was detected at markers D22S1167 (LOD score [Z] 2.09 at recombination fraction [theta] 0) and D22S1154 (Z=1.39 at theta=0), which closely flank the genes for betaB1-crystallin (CRYBB1) and betaA4-crystallin (CRYBA4). Sequencing failed to detect any nucleotide changes in CRYBA4; however, a G-->T transversion in exon 6 of CRYBB1 was found to cosegregate with cataract in the family. This single-nucleotide change was predicted to introduce a translation stop codon at glycine 220 (G220X). Expression of recombinant human betaB1-crystallin in bacteria showed that the truncated G220X mutant was significantly less soluble than wild type. This study has identified the first CRYBB1 mutation associated with autosomal dominant cataract in humans.  (+info)

BetaB1-crystallin: identification of a candidate ciliary body uveitis antigen. (7/70)

PURPOSE: Perineuclear anti-neutrophil cytoplasmic antibody (pANCA), a marker antibody present in 12% of patients with anterior uveitis, recognizes cytoplasmic antigens in the nonpigmented ciliary body epithelium, a probable site of immunologic reactivity in this inflammatory disease. In this study, a recombinantly isolated pANCA monoclonal antibody was used to identify the corresponding antigenic target(s) in the ciliary body. METHODS: Proteins from microdissected eye bank ocular ciliary body tissue were used to identify the corresponding ANCA antigen. Parallel two-dimensional protein gels were used for simultaneous identification of candidate antigenic protein spots by Western blot analysis and as a source of material for proteomic analysis. Multiple independent methods including Western blot analysis, confocal microscopy, and RT-PCR were used to provide additional characterization of the candidate protein. RESULTS: Proteomic analysis suggested that beta B1 (betaB1)-crystallin is the primary ciliary body antigen. The presence of betaB1-crystallin in the human ciliary body was confirmed by Western blot with a betaB1 specific anti-peptide antibody. Confocal microscopy revealed colocalization of the antigenic reactivity of both anti-betaB1 antibody and monoclonal pANCA. RT-PCR confirmed the presence of betaB1-crystallin RNA in the ciliary body tissues. CONCLUSIONS: This study identified betaB1-crystallin as a new cytoplasmic ciliary body antigenic target of a marker autoantibody associated with uveitis. This characterization of betaB1-crystallin outside the lens raises questions about its extralenticular expression, intracellular role, and potential target of inflammation in uveitis.  (+info)

Improving the performance of DomainParser for structural domain partition using neural network. (8/70)

Structural domains are considered as the basic units of protein folding, evolution, function and design. Automatic decomposition of protein structures into structural domains, though after many years of investigation, remains a challenging and unsolved problem. Manual inspection still plays a key role in domain decomposition of a protein structure. We have previously developed a computer program, DomainParser, using network flow algorithms. The algorithm partitions a protein structure into domains accurately when the number of domains to be partitioned is known. However the performance drops when this number is unclear (the overall performance is 74.5% over a set of 1317 protein chains). Through utilization of various types of structural information including hydrophobic moment profile, we have developed an effective method for assessing the most probable number of domains a structure may have. The core of this method is a neural network, which is trained to discriminate correctly partitioned domains from incorrectly partitioned domains. When compared with the manual decomposition results given in the SCOP database, our new algorithm achieves higher decomposition accuracy (81.9%) on the same data set.  (+info)

View mouse Crybb3 Chr5:113075839-113081584 with: phenotypes, sequences, polymorphisms, proteins, references, function, expression
Gene target information for Crybb1 - crystallin, beta B1 (house mouse). Find diseases associated with this biological target and compounds tested against it in bioassay experiments.
A large Indian family consisting of 5 generations containing 33 affected individuals has been described. This is an autosomal dominant disorder in which a mutation has been found in exon 6 of the CRYBB2 gene (22q11.2-q12/22q11.23). This region contains four crystallin genes as well as the CRYBP1 pseudogene. It has been suggested that gene conversion between exon 6 of the CRYBB2 gene and CRYBR1 may be responsible for the phenotype. Three additional families with identical mutations in the same CRYBB2 exon have been reported and, since each family seems to have a unique phenotype, it is likely that more than a simple bp mutation is responsible for the cataracts. Some Coppock-like cataracts (604307) also result from mutations in CRYBB2 at the same location but others have mutations in the CRYGC gene. Type 2 congenital cerulean cataracts (601547) have also been associated with mutations in the CRYBB2 gene. Another autosomal dominant congenital cataract with sutural opacities (600881) has a slightly ...
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CRYBB1 Recombinant Human produced in E.Coli is a single, non-glycosylated polypeptide chain containing 260 amino acids and having a molecular mass of 29.1 kDa.
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References for Abcams Recombinant Human Beta crystallin S protein (ab111642). Please let us know if you have used this product in your publication
Purpose: The purpose of this study was to investigate the impact of three amino acid changes caused by a CRYBB2 gene conversion event that we identified in a large congenital cataract family.. Methods: The CRYBB2 cDNA was generated by RT-PCR using the High Capacity cDNA Reverse Transcription Kit (Applied Biosystems), RNA from human retina, CRYBB2-specific primers and the pcDNA3.1 vector. Three mutations, c.433 C,T (p.R145W), c.440A,G (p.Q147R), and c.449C,T (p.T150M), were introduced into the wild type clone using the QuikChange Lightning kit (Agilent). Cultured human lens epithelium (HLE) SRA 01/04 cells were transfected using Lipofectamine 2000 (Life Technologies). Cell lysates containing protease inhibitors were sonicated and spun to generate separate supernatant and pellet fractions. After SDS-PAGE, Western blot analysis was carried out using a goat polyclonal antibody against CRYBB2 (Santa Cruz Biotechnology).. Results: Missense changes R145W and T150 M were found to be conserved across 15 ...
ABCA4, ABCB6, ABCC6, ABHD12, ACBD5, ADAM9, ADAMTS18, ADGRV1, AGK, AHI1, AIPL1, ALMS1 (excluding exon 8), ARL13B, ARL6, ATF6, B3GLCT, BBS1, BBS10, BBS12, BBS2, BBS4, BBS5, BBS7, BBS9, BCOR, BEST1, BFSP2, BMP4, C19ORF12, C1QTNF5, C2orf71, C8ORF37, CA4, CABP4, CACNA1F, CACNA2D4, CC2D2A, CDH23, CDH3, CDHR1, CEP290, CEP41, CERKL, CFAP410 (C21orf2), CFH, CHM, CHMP4B, CHST6, CIB2, CLN3, CLN5, CLN6, CLN8, CLRN1, CNGA1, CNGA3, CNGB1, CNGB3, CNNM4, COL11A1, COL11A2, COL2A1, COL4A1, COL4A6, COL8A2, COL9A1, COL9A2, CRB1, CRX, CRYAA, CRYAB, CRYBA1, CRYBA4, CRYBB1, CRYBB2, CRYBB3, CRYGB, CRYGC, CRYGD, CRYGS, CTDP1, CTNNA1, CTSD, CYP1B1, CYP4V2, DCN, DHDDS, DSPP, EFEMP1, ELOVL4, EPHA2, EYS, FAM161A, FLVCR1, FRAS1, FREM1, FREM2, FSCN2, FTL, FYCO1, FZD4, GALK1, GALT, GDF3, GDF6, GFER, GIPC3, GJA1, GJA3, GNAT1, GNAT2, GNPTG, GPR143, GPR179, GRIP1, GRK1, GRM6, GRN, GSN, GUCA1A, GUCA1B, GUCY2D, HARS, HCCS, HMX1, HSF4, IDH3B, IFT140, IMPDH1, IMPG1, IMPG2, INVS, ITM2B, IQCB1, JAG1, JAM3, KARS1, KCNJ13, KCNV2, KIF11, ...
Abstract: : Purpose: Pax6, Prox1 and Mafs are transcription factors important for crystallin gene expression and eye development. We previously showed that, in transfection assays, MafB and Prox1 could synergistically activate the chicken betaB1 crystallin promoter (-432/+30), however, both MafB and Prox1 mediated transactivation could be blocked when Pax6 was co-transfected. This study investigates how these transcription factors cooperate to regulate betaB1-crystallin gene expression. Methods: Distribution of MafB was investigated in mouse lens and lens cell lines by immunohistochemistry and western blotting. GST-tagged MafB, Prox1 and Pax6 were generated by using pET or pGEX recombinant protein expression system. Their DNA binding abilities were tested in electrophoresis mobility shift assays (EMSA). Results: MafB was differentially expressed in lens fiber cells and lens epithelial cells: in lens epithelial cells, MafB is not evenly distributed in nuclei but concentrated in a single large ...
α-Conotoxins isolated from Conus venoms contain 11-19 residues and preferentially fold into the globular conformation that possesses a specific disulfide pairing pattern (C1-3, C2-4). We and others isolated a new family of χ-conotoxins (also called λ conotoxins) with the conserved cysteine framework of α-conotoxins but with alternative disulfide pairing (C1-4, C2-3) resulting in the ribbon conformation. In both families, disulfide pairing and hence folding are important for their biological potency. By comparing the structural differences, we identified potential structural determinants responsible for the folding tendencies of these conotoxins. We examined the role of conserved proline in the first intercysteine loop and the conserved C-terminal amide on folding patterns of synthetic analogues of ImI conotoxin by comparing the isoforms with the regiospecifically synthesized conformers. Deamidation at the C-terminus and substitution of proline in the first intercysteine loop switch the ...
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Congenital cataract is a major cause of visual impairment and childhood blindness. The solubility and stability of crystallin proteins play critical roles in maintaining the optical transparency of the lens during the life span. Previous studies have shown that approximately 8.3%∼25% of congenital cataracts are inherited, and mutations in crystallins are the most common. In this study, we attempted to identify the genetic defect in a four-generation family affected with congenital cataracts. The congenital cataract phenotype of this four-generation family was identified as membranous cataract by slit-lamp photography. Mutation screening of the candidate genes detected a heterozygous c.465G→C change in the exon6 of the βB2-crystallin gene (CRYBB2) in all family members affected with cataracts, resulting in the substitution of a highly conserved Tryptophan to Cystine (p.W151C). The mutation was confirmed by restriction fragment length polymorphism (RFLP) analysis and found that the transition
Abstract: β-Crystallins are the major structural proteins existing in the vertebrate lens, and their conformational stability is critical in maintaining the life-long transparency and refraction index of the lens. Seven subunits of β-crystallins naturally assemble into various heteroge-neous oligomers with different sizes. Here, we systematically investigated the thermal sta-bility of the different secondary structures present in β-crystallins and then the dynamic process for the thermal-induced unfolding of β-crystallins by Fourier transform infrared spectroscopy-monitored thermal titration and temperature-jump nanosecond time-resolved IR difference absorbance spectra. Our results show that the N-terminal anti-parallel β-sheets in β-crystallin are the most unstable with a transition midpoint temperature at 36.0±2.1 oC, leading to the formation of an intermediate consisting vastly of random coil structures. This intermediate structure is temporally assigned to that of the monomer ...
Congenital cataract - MedHelps Congenital cataract Center for Information, Symptoms, Resources, Treatments and Tools for Congenital cataract. Find Congenital cataract information, treatments for Congenital cataract and Congenital cataract symptoms.
Purified anti-Alpha Crystallin B - read details of BioLegend antibodies in the SelectScience.net Antibody products and suppliers directory
The current protocol details the preparation of soluble and insoluble protein lysates from mouse brain or spinal cord samples. In detail, tissue homogenization and sequential protein extraction are described. This procedure yields soluble and insoluble protein extracts that can be further processed in down-stream applications like ELISA or Western blotting.
ICD-9 code 743.30 for Congenital cataract unspecified is a medical classification as listed by WHO under the range - CONGENITAL ANOMALIES (740-759).
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Burdon KP, Wirth MG, Mackey DA, Russell-Eggitt IM, Craig JE, Elder JE, Dickinson JL, Sale MM. A novel mutation in the Connexin 46 gene causes autosomal dominant congenital cataract with incomplete penetrance. J Med Genet. 2004 Aug;41(8):e106. Erratum in: J Med Genet. 2005 Mar;42(3):288. J Med Genet. 2008 Apr;45(4):256.. ...
Although multidomain proteins predominate the proteome of all organisms and are expected to display complex folding behaviors and significantly greater structural dynamics as compared with single-domain proteins, their conformational heterogeneity and its impact on their interaction with ligands are poorly understood due to a lack of experimental techniques. The multidomain calcium-binding βγ-crystallin proteins are particularly important because their deterioration and misfolding/aggregation are associated with melanoma tumors and cataracts. Here we investigate the mechanical stability and conformational dynamics of a model calcium-binding βγ-crystallin protein, Protein S, and elaborate on its interactions with calcium. We ask whether domain interactions and calcium binding affect Protein S folding and potential structural heterogeneity. Our results from single-molecule force spectroscopy show that the N-terminal (but not the C-terminal) domain is in equilibrium with an alternative ...
We have investigated the gene expression, effect of deletion from the genome, and the immune response to Acr2, a novel α-crystallin of MTB. MTB has two members of the α-crystallin family of molecular chaperones. Although neither is absolutely required for growth in vivo or in vitro (32), Acr1 has been extensively characterized in the context of its role in the bacterial response to hypoxia/NO and as an immunodominant Ag. Our study demonstrates that Acr2 also makes a contribution to pathogen biology but more so to the host immune response. We found that acr2 gene expression was up-regulated shortly after entry into cells in response to exposure to host reactive oxygen. As a consequence, Acr2 is a prominent immune target, especially of T cells in individuals who are asymptomatic but recently exposed to infectious tuberculosis.. Expression of the acr2 gene is controlled by the heat shock regulator, HspR (5), and by the alternative ς factor, ςE, which is itself regulated by heat shock and by ...
Crystallins are water-soluble proteins that compose about ninety% in the protein inside the lens.[13] The three main crystallin sorts present in the human eye are α-, β-, and γ-crystallins. Crystallins have a tendency to variety soluble, high-molecular pounds aggregates that pack tightly in lens fibers, Therefore raising the index of refraction of the lens while protecting its transparency. β and γ crystallins are found mostly during the lens, while subunits of α -crystallin happen to be isolated from other areas of the eye and the human body ...
Creative Biolabs has established a platform predict and assess deamidation of antibody therapeutics as part of manufacturability assessment.
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Isolation and characterization of a gene expressed mainly in the gastric epithelium, a novel member of the ep37 family that belongs to the βγ-crystallin superfamily (1998 ...
Congenital cataract, although uncommon, accounts for about 10% of childhood blindness.1 The cataract is usually seen as an isolated abnormality but may occur in association with other ocular developmental or systemic abnormalities. About 50% of bilateral cases have a genetic basis. Congenital cataract is both clinically and genetically heterogeneous; isolated congenital cataract is usually inherited as an autosomal dominant trait although autosomal recessive and X linked inheritance are seen less commonly.2 Most progress has been made in identifying the genes causing autosomal dominant congenital cataract.2 Two main approaches have been used to identify the causative mutations. In large families linkage analysis has been used to identify the chromosomal locus followed by screening of positional candidate genes; most genes have been identified using this strategy. A second approach has been to screen DNA from large panels of patients with inherited cataract for mutation in the many candidate ...
TY - JOUR. T1 - βA3/A1-crystallin is required for proper astrocyte template formation and vascular remodeling in the retina. AU - Sinha, Debasish. AU - Valapala, Mallika. AU - Bhutto, Imran. AU - Patek, Bonnie. AU - Zhang, Cheng. AU - Hose, Stacey. AU - Yang, Fang. AU - Cano, Marisol. AU - Stark, Walter J.. AU - Lutty, Gerard A.. AU - Zigler, J. Samuel. AU - Wawrousek, Eric F.. PY - 2012/9. Y1 - 2012/9. N2 - Nuc1 is a spontaneous rat mutant resulting from a mutation in the Cryba1 gene, coding for βA3/A1-crystallin. Our earlier studies with Nuc1 provided novel evidence that astrocytes, which express βA3/A1-crystallin, have a pivotal role in retinal remodeling. The role of astrocytes in the retina is only beginning to be explored. One of the limitations in the field is the lack of appropriate animal models to better investigate the function of astrocytes in retinal health and disease. We have now established transgenic mice that overexpress the Nuc1 mutant form of Cryba1, specifically in ...
Results. Effect of [alpha]-crystallin on restriction enzyme digestion. To determine whether the partially purified [alpha]-crystallin had inhibitory effects on restriction digestion of DNA, we assayed several commonly used restriction enzymes for activity in the presence of [alpha]-crystallin. The commonly used restriction enzymes, Bam HI, Hind III, Nde I, Pst I and Sst I all remained active in the presence of [alpha]-crystallin. For this experiment, a 5 fold excess of each enzyme (5 units) was incubated for 1 hour with 1 µg of plasmid DNA using manufacturers supplied buffer. Each reaction also contained 1 µl of the purified [alpha]-crystallin (10 mg/ml), final concentration, 1 mg/ml. Each selected enzyme digested the plasmid to completion indicating that [alpha]-crystallin had no major detrimental effects on restriction enzyme digestion (data not shown).. To assay the ability of [alpha]-crystallin to protect restriction enzymes from heat inactivation, we chose the enzyme Nde I. Unlike the ...
In the current study, we confirmed a missense mutation c. 139 G , A in Cx50 (GJA8) in a six-generation Chinese pedigree with congenital cataract. This mutation resulted in an asparagine substitution for aspartic at amino acid residue 47 (D47N).. Cataracts are defined as opacification of the normally transparent crystalline lens, and are the leading cause of vision loss in the world. Congenital cataract is a type of cataract that emerges at birth or during early childhood [5, 18]. The abnormality of lens can interfere with normal development of eyes [5, 19]. Congenital cataracts can be inherited or familial, either as an isolated lens phenotype or as part of a genetic/metabolic disorder, commonly with full penetrance and autosomal dominant transmission [19]. Genetic factors play an important role in congenital cataract [20]. Gene mutations that affecting the lens development during embryonic period are considered to be the main cause [18]. Up to now, more than 39 genes and loci have been ...
Pediatric cataracts are observed in 1-15 per 10,000 births with 10-25 % of cases attributed to genetic causes; autosomal dominant inheritance is the most commonly observed pattern. Since the specific cataract phenotype is not sufficient to predict which gene is mutated, whole exome sequencing (WES) was utilized to concurrently screen all known cataract genes and to examine novel candidate factors for a disease-causing mutation in probands from 23 pedigrees affected with familial dominant cataract. Review of WES data for 36 known cataract genes identified causative mutations in nine pedigrees (39 %) in CRYAA, CRYBB1, CRYBB3, CRYGC (2), CRYGD, GJA8 (2), and MIP and an additional likely causative mutation in EYA1; the CRYBB3 mutation represents the first dominant allele in this gene and demonstrates incomplete penetrance. Examination of crystallin genes not yet linked to human disease identified a novel cataract gene, CRYBA2, a member of the βγ-crystallin superfamily. The p.(Val50Met) mutation in ...
A quantitative analysis of cell division and cell elongation was carried out during lens morphogenesis in the rat. At 13 days of development elongating cells in the posterior part of the lens vesicle (presumptive fibre cells) have a lower mitotic activity than cells in the anterior vesicle. By 14 days these elongating cells do not divide. Thus at 14 days of development the lens can be separated into two compartments; a proliferation compartment in the anterior lens and an elongation compartment in the posterior lens.. The three main groups of lens-specific proteins, α-,β- and γ-crystallins, were localized by immunofiuorescence. Alpha-crystallin is the first crystallin to be detected and is localized in some lens pit cells at 12 days of development. By 14 days all lens cells contain α-crystallin. Beta- and β-crystallins are detected later at 12½ days and are localized in some cells situated primarily in the posterior part of the lens vesicle. At later stages of development these crystallins ...
Discussion. The aims of the present study were to comparatively identify parent crystallins of the crystallin fragments present in WS-α-crystallin, WS-HMW, and WI protein fractions of human cataractous (with nuclear opacity) and age matched normal lenses, and to identify post-translational modifications in crystallin species present in the WS-HMW and WI protein fractions of cataractous lenses. The rationale was that the results would distinguish cataract specific aggregated crystallin species from those that aggregate during aging in both WS-HMW and WI protein fractions.. The major findings of this study are: (1) the majority of aggregated species in the WS-HMW and in the WI protein fractions during aging and nuclear cataract development were fragments of αA- and αB-crystallin, (2) while the younger lenses contained only the C-terminally truncated αA- and αB-crystallin, the older normal lenses showed age related truncation of both N- and C- terminal regions in the crystallins, (3) in the ...
Cataract is a major form of blindness that occurs when the structure of crystallin proteins that make up the lens in our eyes deteriorates, causing damaged or disorganised proteins to aggregate and form a milky blue or brown layer, which ultimately affects lens transparency ...
The major components of the lens are highly stable and water-soluble crystallin proteins. Once deposed, the proteins remain in the lens and create a structure providing unique transparency and accommodation properties [12]. In spite of the high stability, molecular changes accumulate in the proteins over time caused by chemical, photochemical, environmental factors or by ageing and concomitant diseases, e.g. diabetes [13,14]. The changes lead to protein misfolding, denaturation and aggregation and hence structural defects [15]. Currently, cataract diagnostics based on light scattering only detect defects comparable in size to the wavelength so that detection of the impact of photochemical change is hardly possible at the stage when a cataract is currently diagnosed. Thus, surgical treatment is the only currently possible method.. Although cataract surgery is generally recognized as being one of the safest operations, and there is a significant complication rate [16]. Furthermore, there is ...
de Jong, W.W., Zweers, A. and Goodman, M. (1981) Relationship of Aardvark to Elephants, Hyraxes and Sea Cows from α-Crystallin Sequences. Nature, 292, 538-540.
The outcome of treatment for bilateral congenital cataracts was studied retrospectively in a group of 51 patients. Two major categories of lens opacities were identified. In the first category, the opacities were extensive and visual impairment was evident early in the first year. These cataracts of …
During development of the mammalian eye, nourishment of the immature lens, inner retina and vitreous is provided by the hyaloid vascular system. One of the cong...
Cataracts present from birth (congenital cataracts) are sometimes caused by a faulty gene being passed to a child from their parents. This fault means that the lens doesnt develop properly.. Its estimated that theres a family history of congenital cataracts in around one in every five cases of the condition, and recent research suggests genetic causes are responsible for the majority of bilateral congenital cataracts in the UK.. Cataracts can also be associated with conditions caused by chromosome abnormalities, such as Downs syndrome. Chromosomes are the parts of the bodys cells that carry the genes. ...
The long-term objective of this proposal is to determine the molecular mechanisms by which modifications of the ?-crystallins (genetic or age-onset), lead to ca...
When a baby is born with cataracts or develops them at a very early age, the condition is called congenital or infantile cataracts. A cataract can make your vision blurry, like trying to look through frosted glass.
Open-angle glaucoma - Congenital cataract Factor. Last reviewed for CCPS 18 July 2006.. Preliminary questions [28822]. 31527 [1] there is some evidence that congenital cataract may be a factor in the development of the condition under consideration.. 28823 - the veteran satisfies the preliminary requirements for claiming congenital cataract as the cause of the condition under consideration.. 11996 [2] the condition under consideration is angle-closure glaucoma.. or. 11997 [3] the condition under consideration is open-angle glaucoma.. 11998 [4] the veterans open-angle glaucoma is a phacolytic glaucoma.. [5]28155. the veteran has suffered from a congenital cataract at some time.. 28824 [1] the congenital cataract affected side and site of the body at some time.. [6]28825. the congenital cataract was affecting side and site of the body at the time of the clinical onset of the condition under consideration. [7]. 28826 - the veteran has established the causal connection between the congenital ...
Human ζ-crystallin is a Zn(2+)-lacking medium-chain dehydrogenase/reductase (MDR) included in the quinone oxidoreductase (QOR) family because of its activity with quinones. In the present work a novel enzymatic activity was characterized: the double bond α,β-hydrogenation of medium-chain 2-alkenals and 3-alkenones. The enzyme is especially active with lipid peroxidation products such as 4-hydroxyhexenal, and a role in their detoxification is discussed. This specificity is novel in the QOR family, and it is similar to that described in the distantly related alkenal/one reductase family. Moreover, we report the X-ray structure of ζ-crystallin, which represents the first structure solved for a tetrameric Zn(2+)-lacking MDR, and which allowed the identification of the active-site lining residues. Docking simulations suggest a role for Tyr53 and Tyr59 in catalysis. The kinetics of Tyr53Phe and Tyr59Phe mutants support the implication of Tyr53 in binding/catalysis of alkenal/one substrates, while Tyr59 is
Dr. Mason Posner has students use molecular biology techniques to understand how eye lens proteins adapt to changes in environmental temperature.. We are currently investigating the evolution and biological role of lens proteins called crystallins. These proteins are responsible for making the lens transparent and refracting light so that focused images fall on the retina. Amazingly, one family of crystallins, the alpha crystallins, also protect other proteins from the harmful effects of aging that can lead to lens cataracts, one of the leading causes of blindness in humans. Alpha crystallins are also involved in the original development of the lens in vertebrate embryos, and they have been linked to many diseases of the nervous system, heart, skeletal muscle, and are now known to be involved in many cancers.. Most research into alpha crystallins is done with mammals. However, by studying how this protein has evolved in a number of fish species that live at different environmental temperatures, ...
Inhibin beta B antibody [C3], C-term (inhibin, beta B) for ICC/IF, IHC-P, WB. Anti-Inhibin beta B pAb (GTX108408) is tested in Human samples. 100% Ab-Assurance.
11- Zafaranchi zadeh moghadam S, Khoshaman K, Masoudi R, Hemmateenejad B, Yousefi R (2017) The structural alteration and aggregation propensity of glycated lens crystallins in the presence of calcium; importance of lens calcium homeostasis in development of diabetic cataracts. Spectrochimica Acta Part A: Molec Spectrochim Acta A Mol Biomol Spectroscopy 170:174-183 ...
Expression of insoluble protein - posted in Protein Expression and Purification: Hi AllI am facing a serious problem in expression of a protein in soluble fractionI have a full-length gene (approx. 1.6kb) cloned in pExp5 TOPO/ CT vector.Sequencing of the clones revealed that the gene is in-frame. I have expressed it in Rosetta-BL21(DE3)pLys-S cells at 18 C in an autoinduction media. I could see the clear expression in lysed cell pellate (~64KDa) when SDS-PAGE was done, whereas the final el...
Learn about the causes, symptoms, diagnosis & treatment of Eye Disorders in Children from the Home Version of the Merck Manuals.
CRYM antibody [1G7] (crystallin, mu) for FACS, ICC/IF, IHC-P, WB. Anti-CRYM mAb (GTX84654) is tested in Human, Rat samples. 100% Ab-Assurance.
Author Kristin Newman shares a few of her favourite ways to spend a day near Four Seasons destinations, from Los Angeles to Chiang Mai and beyond.
Mammalian lens crystallins are divided into alpha, beta, and gamma families; beta and gamma crystallins are also considered as ... Alpha-crystallin A chain is a protein that in humans is encoded by the CRYAA gene. Crystallins are separated into two classes: ... Alpha and beta families are further divided into acidic and basic groups. Seven protein regions exist in crystallins: four ... de Jong WW, Terwindt EC, Bloemendal H (1976). "The amino acid sequence of the A chain of human alpha-crystallin". FEBS Lett. 58 ...
"Autosomal dominant cerulean cataract is associated with a chain termination mutation in the human beta-crystallin gene CRYBB2 ... This gene, a beta basic group member, is part of a gene cluster with beta-A4, beta-B1, and beta-B3. A chain-terminating ... "Linkage between the beta B2 and beta B3 crystallin genes in man and rat: a remnant of an ancient beta-crystallin gene cluster ... Mammalian lens crystallins are divided into alpha, beta, and gamma families; beta and gamma crystallins are also considered as ...
... alpha-crystallin b chain MeSH D12.776.306.366.300.100 - beta-crystallin a chain MeSH D12.776.306.366.300.200 - beta-crystallin ... myosin heavy chains MeSH D12.776.210.500.600.200 - myosin light chains MeSH D12.776.210.500.600.300 - myosin subfragments MeSH ... myosin heavy chains MeSH D12.776.220.525.475.200 - myosin light chains MeSH D12.776.220.525.475.300 - myosin subfragments MeSH ... alpha-crystallin a chain MeSH D12.776.306.366.100.300 - ... beta-2 microglobulin MeSH D12.776.377.715.182.160 - beta- ...
Mammalian lens crystallins are divided into alpha, beta, and gamma families; beta and gamma crystallins are also considered as ... Alpha-crystallin B chain is a protein that in humans is encoded by the CRYAB gene. It is part of the small heat shock protein ... Alpha B chain crystallins (αBC) can be induced by heat shock, ischemia, and oxidation, and are members of the small heat shock ... Alpha and beta families are further divided into acidic and basic groups. Seven protein regions exist in crystallins: four ...
... rho crystallin, and many others. All possess a similar structure, with a beta-alpha-beta fold characteristic of nucleotide ... Some proteins of this family contain a potassium channel beta chain regulatory domain; these are reported to have ... The binding site is located in a large, deep, elliptical pocket in the C-terminal end of the beta sheet, the substrate being ... Gulbis JM, Zhou M, Mann S, MacKinnon R (July 2000). "Structure of the cytoplasmic beta subunit-T1 assembly of voltage-dependent ...
... alpha 1 Crystallin, beta A1 Cyclic nucleotide-gated channel alpha 1 Cyclin A1 Cytochrome P450, family 1, member A1 Defensin, ... a portion of the cholera toxin chain alleles A1, an allele in the DRD2 TaqI polymorphism that could be involved in alcoholism. ... DQ alpha 1 Myosin light chain A1, an actin-binding protein NADH dehydrogenase (ubiquinone), alpha 1 Nucleolar protein, member ...
Crystallin zeta CYP4B1 (1p33) CYR61 (1p22) DBT: dihydrolipoamide branched chain transacylase E2 DCLRE1B: DNA cross-link repair ... beta; acid (includes glucosylceramidase) (gene for Gaucher disease) GBAP1: glucosylceramidase beta pseudogene 1 GLC1A: gene for ... beta 3, 31kDa (connexin 31) GLMN (1p22) GNL2: G protein nucleolar 2 GSTM1 (1p13) HDAC1 (1p35) HES2: Hes family bHLH ... heavy chain 14 ECM1 (1q21) EDEM3: ER degradation enhancing alpha-mannosidase like protein 3 EGLN1 (1q42) ENAH (1q42) ESRRG ( ...
The yeast killer toxin structure was thought to be a precursor of the two-domain beta gamma-crystallin proteins, because of its ... is a double-chain inhibitor consisting of an 11-residue and a 41-residue chain. The Carboxypeptidase inhibitor I68 family ... with two alpha-helices and four beta-strands with a (beta/alpha/beta)x2 topology. The peptidase inhibitor I9 family contains ... SMPI is composed of two beta-sheets, each consisting of four antiparallel beta-strands. The structure can be considered as two ...
4-beta-glucosidase MeSH D08.811.277.450.420.200.600 - glucan endo-1,3-beta-d-glucosidase MeSH D08.811.277.450.420.375 - glucan ... cholesterol side-chain cleavage enzyme MeSH D08.811.600.250 - electron transport chain complex proteins MeSH D08.811.600.250. ... delta-crystallins MeSH D08.811.520.232.400 - ammonia-lyases MeSH D08.811.520.232.400.200 - aspartate ammonia-lyase MeSH D08.811 ... 4-beta-cellobiosidase MeSH D08.811.277.450.420.200.450 - endo-1,3(4)-beta-glucanase MeSH D08.811.277.450.420.200.500 - glucan 1 ...
CRYZ: Crystallin zeta. *CYP4B1 (1p33). *CYR61 (1p22). *DBT: dihydrolipoamide branched chain transacylase E2 ... GBA: glucosidase, beta; acid (includes glucosylceramidase) (gene for Gaucher disease). *GBAP1: glucosylceramidase beta ... ACADM: acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain ... GJB3: gap junction protein, beta 3, 31kDa (connexin 31). *GLMN ...
The translation of reticulocyte 9S RNA in frog oocytes gives rise to alpha and beta globin chains. Communication to FEBS ... Berns, A. J. M.; Kraaikamp, M. Van; Bloemendal, H.; Lane, C. D. (1972). "Calf Crystallin Synthesis in Frog Cells: The ...
"Myxococcus xanthus spore coat protein S may have a similar structure to vertebrate lens beta gamma-crystallins". Nature. 315 ( ... However, these binding sites were based on a cluster analysis of side-chain oxygen atoms and on results from site-directed ... "Structural similarity of a developmentally regulated bacterial spore coat protein to beta gamma-crystallins of the vertebrate ... The domains share high similarity with other crystallin proteins. Protein S binds two 2 mol of calcium per mol of protein with ...
Also in the 1970s, physicists Tanaka & Benedek at MIT identified phase-separation behaviour of gamma-crystallin proteins from ... Schaefer KN, Peifer M (February 2019). "Wnt/Beta-Catenin Signaling Regulation and a Role for Biomolecular Condensates". ... chains linked by covalent bonds), which he termed 'macromolecules' to distinguish them from colloidal clusters of lower ... Schaefer KN, Peifer M (February 2019). "Wnt/Beta-Catenin Signaling Regulation and a Role for Biomolecular Condensates". ...
"Induction of tau pathology by intracerebral infusion of amyloid-beta -containing brain extract and by amyloid-beta deposition ... Ecroyd H, Carver JA (January 2009). "Crystallin proteins and amyloid fibrils". Cellular and Molecular Life Sciences. 66 (1): 62 ... Badar T, D'Souza A, Hari P (2018). "Recent advances in understanding and treating immunoglobulin light chain amyloidosis". ... Wisniewski HM, Sadowski M, Jakubowska-Sadowska K, Tarnawski M, Wegiel J (July 1998). "Diffuse, lake-like amyloid-beta deposits ...
δ-crystallins are the major structural eye lens water-soluble proteins of most birds, reptiles, and some other vertebrates. ... Domain 2 contains one small beta sheet, nine alpha helices, and the carboxyl terminus. Three of the nine alpha helices on one ... ASL is composed of four identical monomers; each monomer consisting of a single polypeptide chain between 49 and 52 kDa, ... ASL, δ-crystallin, class II fumarase, aspartase, adenylosuccinase lyase, and 3-carboxy-cis and cis-muconate lactonizing enzyme ...
Beta cell (secretes insulin and amylin) Delta cell (secretes somatostatin) Epsilon cell (secretes ghrelin) PP cell (gamma cell ... Intermediate skeletal muscle cell Nuclear bag cell of muscle spindle Nuclear chain cell of muscle spindle Myosatellite cell ( ... Medium spiny neurons Astrocytes Oligodendrocytes Ependymal cells Tanycytes Pituicytes Anterior lens epithelial cell Crystallin- ...
The N-terminal domain I (aa ~1-195) contains attachment sites for HS chains. Although HS chains are not required for correct ... This role for perlecan in beta-amyloid plaque generation is supported by an earlier study showing that perlecan and beta- ... To this end, a transgenic mouse line was created expressing porcine TGF-β1 under the lens-specific αA-crystallin promoter and ... Domain V also has attachment sites for HS/CS chains. Thus, perlecan core protein and HS chains could modulate matrix assembly, ...
Silva, R.A.G.D., *Nguyen, J.Y., and Decatur, S.M. (2002) "Probing the Effects of Side Chains on the Conformation and Stability ... Phi Beta Kappa (elected 1990) Sigma Xi (elected 1990) Op-ed pieces on higher education "The Winner - A Liberal Education," New ... "Formation of Amyloid Fibrils in Vitro by Human γd-crystallin and its Isolated Domains," Molecular Vision 14:81-89. Decatur, S.M ... "Twodimensional IR Spectroscopy and Segmental 13C Labeling reveals the Domain Structure of Human γδ-crystallin amyloid fibrils ...
... a proline occurs at position 67 in the chain of amino acids that make up the A2 beta-casein, while in A1 beta-casein a ... Milk Casein and Lens Crystallin Proteins", Protein Nanotechnology, Totowa, NJ: Humana Press, 996, pp. 103-117, doi:10.1007/978- ... beta-casomorphin-7, (BCM-7) can be released by digestion of A1-beta-casein. The A1 beta-casein type is the most common type ... A1 and A2 beta-casein are genetic variants of the beta-casein milk protein that differ by one amino acid; ...
Oligomerization is a chemical process that converts individual molecules into a chain consisting of a finite number of ... "Monitoring the prevention of amyloid fibril formation by alpha-crystallin. Temperature dependence and the nature of the ... Amyloid beta can be measured semiquantitatively with immunostaining, which also allows one to determine location. Amyloid beta ... By NMR-guided simulations, amyloid beta 1-40 and amyloid beta 1-42 also seem to feature highly different conformational states ...
free light chains (FLC). Several authors have reported that they are comparable or even better than oligoclonal bands. Multiple ... Bsibsi M, Holtman IR, Gerritsen WH, Eggen BJ, Boddeke E, van der Valk P, van Noort JM, Amor S (2013). "Alpha-B-Crystallin ... in patients with relapsing-remitting multiple sclerosis treated with interferon beta". Clin Neurol Neurosurg. 108 (2): 124-8. ...
Iodine (I0) is very reactive and iodinates the thyroglobulin at tyrosyl residues in its protein chain (in total containing ... However, recently it could be demonstrated that the crystallin CRYM binds 3,5,3′-triiodothyronine in vivo.[37] ... "Genome-wide binding patterns of thyroid hormone receptor Beta". PLOS One. 9 (2): e81186. Bibcode:2014PLoSO...981186A. doi ... "μ-Crystallin as an Intracellular 3,5,3′-Triiodothyronine Holder in Vivo". Molecular Endocrinology. 21 (4): 885-894. doi ...
Wang P, Gao H, Ni Y, Wang B, Wu Y, Ji L, Qin L, Ma L, Pei G (February 2003). "Beta-arrestin 2 functions as a G-protein-coupled ... Wang P, Wu Y, Ge X, Ma L, Pei G (March 2003). "Subcellular localization of beta-arrestins is determined by their intact N ... In addition, MDM2 has p53-independent transcription factor-like effects in nuclear factor-kappa beta (NFκB) activation. ... Long-chain-fatty-acid-CoA ligase. 6.3: Carbon-Nitrogen. *Glutamine synthetase. *Ubiquitin ligase *Cullin ...
The cause is lens hardening by decreasing levels of alpha-crystallin, a process which may be sped up by higher temperatures. ... The authors propose that mtDNA mutations lead to respiratory-chain-deficient cells and thence to apoptosis and cell loss. They ... "Age-Related Loss of Innate Immune Antimicrobial Function of Dermal Fat Is Mediated by Transforming Growth Factor Beta". ... the Growth hormone/Insulin-like growth factor 1 signalling pathway the activity levels of the electron transport chain in ...
α-crystallin, a process which may be sped up by higher temperatures.[19][20] ... The authors propose that mtDNA mutations lead to respiratory-chain-deficient cells and thence to apoptosis and cell loss. They ... "Age-Related Loss of Innate Immune Antimicrobial Function Of Dermal Fat Is Mediated By Transforming Growth Factor Beta" ... the activity levels of the electron transport chain in mitochondria[57] and (in plants) in chloroplasts. ...
Truncated human betaB1-crystallin shows altered structural properties and interaction with human betaA3-crystallin. ... The purpose of the study was to determine the effects of truncation of various regions of betaB1-crystallin on its structural ... properties and stability of heterooligomers formed by wild-type (WT) betaB1 or its deletion mutants with WT betaA3-crystallin. ...
Alpha-crystallin A2 chainAdd BLAST. 173. ChainiPRO_0000444508. 1 - 172. Alpha-crystallin A2(1-172)By similarity. ,p>Manually ... Heat shock protein beta-4. MACMU. 210. +9. A0A140G945-2. SHSP domain-containing protein. PANTR ... Chain Alpha-crystallin A2 chain:. Molecular mass is 20029 Da. Determined by ESI. With 1 phosphate group.1 Publication. Manual ... Chain Alpha-crystallin A2 chain:. Molecular mass is 19950 Da. Determined by ESI. 1 Publication. Manual assertion based on ...
Mouse Monoclonal Anti-AlphaA Crystallin/CRYAA Antibody (1H3.B8). Validated: WB, ELISA, IB, ICC/IF. Tested Reactivity: Human. ... Heat shock protein beta-4. *HSPB4. *HSPB4alpha-crystallin A chain. *human alphaA-crystallin (CRYA1), 10HspB4 ... Does not cross-react with aB-crystallin, Beta L-crystallin, BH- crystallin, gamma-crystallin, HSP25, HSP27 or HSP47 proteins. ... Home » AlphaA Crystallin/CRYAA » AlphaA Crystallin/CRYAA Antibodies » AlphaA Crystallin/CRYAA Antibody (1H3.B8) ...
Crystallin Beta A1, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene ... Beta Crystallin A3 Chain Transcript LAM 3 * Beta Crystallin A3 Chain Transcript CN 3 ... crystallin, beta A3 and crystallin, beta A1) from a single mRNA, the latter protein is 17 aa shorter than crystallin, beta A3 ... Mammalian lens crystallins are divided into alpha, beta, and gamma families; beta and gamma crystallins are also considered as ...
Suppression of thermal aggregation of beta-L-crystallin by alpha-crystallin. Biochemistry 2005, 44, 15480-15487. [Google ... These light chains are nearly identical except for first 41 additional amino acid residues of the A1 light chain, containing ... Interaction of the N-terminal part of the A1 essential light chain with the myosin heavy chain. Biochem. Biophys. Res. Commun ... also called alkali light chain [4]. In fast skeletal muscle myosin there are two kinds of alkali light chains, A1 and A2. ...
Autosomal dominant cerulean cataract is associated with a chain termination mutation in the human beta-crystallin gene CRYBB2. ... Three main families of mammalian crystallins are α-, β-, and γ-crystallins. α-Crystallins (αA and αB) are homologous to small ... Crystallins of the beta/gamma-superfamily mimic the effects of lens injury and promote axon regeneration. Mol Cell Neurosci. ... The evolution of lenticular proteins: the beta- and gamma-crystallin super gene family. Prog Biophys Mol Biol. 1988;51:47-76. [ ...
Chain. 2 - 174. Gamma-crystallin D. Domain. 88 - 128. Beta/gamma crystallin Greek key 3. ... Structural and evolutionary relationships among five members of the human gamma-crystallin gene family. Meakin S.O.; Breitman M ... Novel mutations in the gamma-crystallin genes cause autosomal dominant congenital cataracts. Santhiya S.T.; Shyam Manohar M.; ...
Rat A crystallin A Monoclonal Antibody (Clone c9F2)-NP_000385 (MBS200007) product datasheet at MyBioSource, Primary Antibodies ... Heat shock protein beta-4; HspB4; Alpha-crystallin A chain; Heat shock protein beta 4; Acry-1; Alpha crystallin A chain; CRYA 1 ... Mammalian lens crystallins are divided into alpha, beta, and gamma families. Alpha crystallins are composed of two gene ... This antibody is not shown cross-activity about Crystallin alpha B. A : Crystallin-alpha A recombinant protein B : Crystallin- ...
Alpha crystallin B chain; Alpha crystallin B chain; Alpha(B) crystallin; Alpha(B)-crystallin; Alpha-crystallin B chain; CRYA2; ... Mammalian lens crystallins are divided into alpha, beta, and gamma families; beta and gamma crystallins are also considered as ... β5/α ״???? B/αB-crystallin ??. ??. alpha B Crystallin (phospho S53); alpha B Crystallin (phospho Ser53); p-alpha B Crystallin ( ... Alpha and beta families are further divided into acidic and basic groups. Seven protein regions exist in crystallins: four ...
Rabbit polyclonal alpha A Crystallin/CRYAA antibody. Validated in WB, IHC, BL, sELISA and tested in Mouse, Rat, Cow. Cited in ... The mammalian lens contains 3 major classes of crystallins: alpha, beta, and gamma. Alpha-crystallin is the largest of the ... O-glycosylated; contains N-acetylglucosamine side chains.. Deamidation of Asn-101 in lens occurs mostly during the first 30 ... There are at least 5 different proteins comprising the beta-crystallins. The gamma-crystallins are monomeric, but there are at ...
... wherein a rearrangement of the amino terminal of the beta-chain of hemoglobin by reaction with glucose, forms the adduct known ... This reaction was also found to occur with a variety of other body proteins, such as lens crystallins, collagen and nerve ... The preferred are peroxidase, beta-glucuronidase, beta-D-glucosidase, beta-D-galactosidase, urease, glucose oxidase plus ... The indicator composition of claim 14 wherein said label is selected from peroxidase, beta-glucuronidase, beta-D-glucosidase, ...
Autosomal dominant cerulean cataract is associated with a chain termination mutation in the human beta-crystallin gene CRYBB2. ... A unique form of autosomal dominant cataract explained by gene conversion between beta-crystallin B2 and its pseudogene. J Med ... The γ-crystallin gene cluster on chromosome 2q33-35 encompasses genes γA to D but only γC (CRYGC) and γD(CRYGD) are highly ... Heon E, Priston M, Schorderet DF, et al. The gamma-crystallins and human cataracts: a puzzle made clearer. Am J Hum Genet 1999; ...
Rabbit polyclonal Alpha B Crystallin (phospho S19) antibody validated for WB, IHC, ICC/IF and tested in Human, Mouse and Rat. ... Alpha crystallin B chain antibody. *Alpha(B)-crystallin antibody. *Alpha-crystallin B chain antibody ... Heat shock protein beta 5 antibody. *Heat shock protein beta-5 antibody ... Lane 1 : phospho-alpha-B crystallin (Ser19). Lane 2 : unphosphorylated alpha-B crystallin. Observed band size : 20 kDa (why is ...
T-cell receptor beta-chain (38), 2-crystallin (39), 1 tubulin The functional and physical unit of heredity passed from parent ... T-cell receptor beta-chain enhancer: the role of enhancer elements in controlling V(D)J recombination accessibility. Proc Natl ... differentiation stage-specific expression of the rat beta B2-crystallin The functional and physical unit of heredity passed ... differentiation stage-specific expression of the rat beta B2-crystallin The functional and physical unit of heredity passed ...
Mammalian lens crystallins are divided into alpha, beta, and gamma families; beta and gamma crystallins are also considered as ... Alpha-crystallin A chain is a protein that in humans is encoded by the CRYAA gene. Crystallins are separated into two classes: ... Alpha and beta families are further divided into acidic and basic groups. Seven protein regions exist in crystallins: four ... de Jong WW, Terwindt EC, Bloemendal H (1976). "The amino acid sequence of the A chain of human alpha-crystallin". FEBS Lett. 58 ...
1997 Autosomal dominant cerulean cataract is associated with a chain termination mutation in the human beta-crystallin gene ... 2008 Crystallin gene mutations in Indian families with inherited pediatric cataract. Mol. Vis. 14: 1157-1170. ... 2004b Characterization of the G91del CRYBA1/3-crystallin protein: a cause of human inherited cataract. Hum. Mol. Genet. 13: 945 ... 1999 The gamma-crystallins and human cataracts: a puzzle made clearer. Am. J. Hum. Genet. 65: 1261-1267. ...
beta-Crystallin A Chain/biosynthesis. *beta-Crystallin A Chain/genetics*. PubMed:. 23508780 Full text @ Hum. Genet. ... Examination of crystallin genes not yet linked to human disease identified a novel cataract gene, CRYBA2, a member of the βγ- ... crystallin superfamily. The p.(Val50Met) mutation in CRYBA2 cosegregated with disease phenotype in a four-generation pedigree ...
Purified anti-Alpha Crystallin B - read details of BioLegend antibodies in the SelectScience.net Antibody products and ... Alpha-crystallin B chain, alpha B crystallin, heat shock protein beta-5, rosenthal fiber component, heat-shock 20 kD like- ... Purified anti-Alpha Crystallin B. Be the first to review this product ... Purified anti-Alpha Crystallin B; Clone: Poly9079; Reactivity: Human, Mouse; Apps: IHC, WB; Size: 200 μl ...
... non-glycosylated polypeptide chain containing 216 amino acids (1-196 a.a.). ... Beta-crystallin A4 (CRYBA4) is a member of the beta/gamma-crystallin family which are the dominant structural components of the ... The CRYBA4 gene being a beta acidic group member is part of a gene cluster with beta-B1, beta-B2, and beta-B3. ... Beta-crystallins construct aggregates of various sizes and are able to self-associate to form dimers or to form heterodimers ...
... non-glycosylated polypeptide chain containing 260 amino acids and having a molecular mass of 29.1 kDa. ... EC 1.17.4.1, RR2M, Beta-B1 crystallin, CATCN3.. Introduction. Crystallins are the main structural proteins of the vertebrate ... Crystallins are divided into 3 fprotein families, α, β, & γ families. Because lens central fiber cells lose their nuclei during ... CRYBB1 is a beta basic group member and undergoes extensive cleavage at its N-terminal extension during lens maturation. ...
Linkage of the locus encoding the A chain of alpha-crystallin (Acry-1) to the major histocompatibility complex in the rat. Skow ... Hematology of a murine beta-thalassemia: a longitudinal study. Popp RA, Popp DM, Johnson FM, Skow LC, Lewis SE. Ann N Y Acad ... Polymorphism and linkage of the alpha A-crystallin gene in t-haplotypes of the mouse. Skow LC, Nadeau JN, Ahn JC, Shin HS, ... The locus encoding alpha A-crystallin is closely linked to H-2K on mouse chromosome 17. Skow LC, Donner ME. Genetics. 1985 110( ...
... alpha-crystallin b chain MeSH D12.776.306.366.300.100 - beta-crystallin a chain MeSH D12.776.306.366.300.200 - beta-crystallin ... myosin heavy chains MeSH D12.776.210.500.600.200 - myosin light chains MeSH D12.776.210.500.600.300 - myosin subfragments MeSH ... myosin heavy chains MeSH D12.776.220.525.475.200 - myosin light chains MeSH D12.776.220.525.475.300 - myosin subfragments MeSH ... alpha-crystallin a chain MeSH D12.776.306.366.100.300 - ... beta-2 microglobulin MeSH D12.776.377.715.182.160 - beta- ...
... alpha crystallin B chain (3.3fold, p=0.0001) and beta crystallin B2 (2.99fold, p=0.00013). ...
... λ and κ L chains, and albumin, whereas albumin, IgG1 H chain, and λ and κ L chains were observed in samples from normal sera ( ... Antibodies to myelin basic protein, myelin oligodendrocytes peptides, alpha-beta-crystallin, lymphocyte activation and cytokine ... Crystal structures of alpha-crystallin domain dimers of alphaB-crystallin and Hsp20. J. Mol. Biol. 392: 1242-1252. ... Natural beta-sheet proteins use negative design to avoid edge-to-edge aggregation. Proc. Natl. Acad. Sci. USA 99: 2754-2759. ...
beta-Crystallin A Chain / genetics Actions. * Search in PubMed * Search in MeSH ... Genetics of crystallins: cataract and beyond. Graw J. Graw J. Exp Eye Res. 2009 Feb;88(2):173-89. doi: 10.1016/j.exer.2008.10. ... Novel mutation in the gamma-S crystallin gene causing autosomal dominant cataract. Vanita V, Singh JR, Singh D, Varon R, ... A novel MAF missense mutation leads to congenital nuclear cataract by impacting the transactivation of crystallin and ...
beta-Crystallin A Chain / genetics* Actions. * Search in PubMed * Search in MeSH ... Association of high myopia with crystallin beta A4 (CRYBA4) gene polymorphisms in the linkage-identified MYP6 locus Daniel W H ... Association of high myopia with crystallin beta A4 (CRYBA4) gene polymorphisms in the linkage-identified MYP6 locus Daniel W H ... Based on initial and replication studies, rs2009066 located in the crystallin beta A4 (CRYBA4) gene was identified to be the ...
Alpha-crystallin A chain. Alpha-crystallin A chain (Heat shock protein beta-4, HspB4) [Cleaved into: Alpha-crystallin A(1-172) ... Beta-ketoacyl reductase) ; 3-oxoacyl-[acyl-carrier-protein] synthase, EC 2.3.1.41 (Beta-ketoacyl synthase) ] ...
Genes of interest are cloned into the multiple cloning site of the Vector System just downstream of the p26 or alpha crystallin ... A method for expressing proteins as a fusion chimera with a domain of p26 or alpha crystallin type proteins to improve the ... As used herein the active anti-parallel beta sheets is defined by amino acid residues 36-160. As used herein the term " ... Hayhurst, A., "Improved Expression Characteristics of Single-Chain Fv Fragments When Fused Downstream of the Escherichia coli ...
... beta-myosin heavy chain was upregulated (+1.7). In the RA, 22 proteins spots were altered including the following downregulated ... Crystallin alpha B (hypertrophy inhibitor) was upregulated in both the RV (+2.2) and RA (+2.6).In early stage hypertrophy there ... Downregulated RV proteins included contractile elements: troponin T and C (-1.6 fold change), myosin regulatory light chain 2 ... proteins contractile elements: tropomyosin 1 alpha chain (-1.9), cellular energetic proteins: ATP synthase (-1.5), fatty-acid ...
aml:100476659 crystallin beta B1 283 104 ( -) 30 0.368 68 -, aml:100477329 inhibitor of growth family member 4 K11346 248 104 ... aml:100478914 collagen type IV alpha 4 chain K06237 1676 320 ( -) 79 0.271 469 -, aml:100480195 collagen type IV alpha 5 chain ... aml:100468912 collagen type XI alpha 1 chain K19721 1823 271 ( -) 68 0.246 472 -, aml:100474885 collagen type I alpha 2 chain ... aml:100478752 collagen type IV alpha 1 chain K06237 1643 277 ( -) 69 0.261 406 -, aml:100484506 GLI family zinc finger 2 K16798 ...
  • 8 9 A number of studies reported the upregulation of crystallin genes in various tissues in response to stress or pathologic conditions. (arvojournals.org)
  • Novel mutations in the gamma-crystallin genes cause autosomal dominant congenital cataracts. (expasy.org)
  • The γ-crystallin gene cluster on chromosome 2q33-35 encompasses genes γ A to D but only γ C (CRYGC) and γ D (CRYGD) are highly expressed in the human lens. (bmj.com)
  • 10 The β-crystallin family encompasses four acidic (A) and three basic (B) forms encoded by genes on chromosomes 2, 17, and 22. (bmj.com)
  • Four mutations have been reported in the β-crystallin genes. (bmj.com)
  • At least 15 different mutations in the crystallin genes have now been implicated in human cataract associated with a diverse range of phenotypes. (bmj.com)
  • It is still unclear what proportion of inherited cataract is associated with crystallin gene mutations as few studies have involved systematic screening of all crystallin genes in a large patient population. (bmj.com)
  • Approximately one-half of the mutations are in the crystallin genes, and one-quarter are in the connexin genes. (g3journal.org)
  • Examination of crystallin genes not yet linked to human disease identified a novel cataract gene, CRYBA2, a member of the βγ-crystallin superfamily. (zfin.org)
  • Genes of interest are cloned into the multiple cloning site of the Vector System just downstream of the p26 or alpha crystallin type protein and a thrombin cleavage site. (google.com)
  • Because of their high levels of expression, crystallins represent compelling candidate genes for inherited cataracts. (molvis.org)
  • Different mutations in crystallin genes could lead to distinctive cataract phenotypes. (molvis.org)
  • Results: From the large data set generated, selected candidate genes were confirmed by reverse transcriptase-polymerase chain reaction (RT-PCR) and in situ hybridization. (uwindsor.ca)
  • Conclusions: We have identified four genes as likely direct targets of Pitx3 action: Pax6, beta Crystallin-b1 (Crybb1), Hes7.1, and Hes4. (uwindsor.ca)
  • Purpose: Identification of causal mutation in the crystallin, connexin, and paired box 6 (PAX6) genes associated with childhood cataract in patients from India. (elsevier.com)
  • crystallin genes: A gene family on its way to extinction. (springer.com)
  • Immunocytochemistry/ Immunofluorescence: AlphaA Crystallin/CRYAA Antibody (1H3.B8) [NBP2-12875] - Tissue: Neuroblastoma cell line SK-N-BE. (novusbio.com)
  • 3 α-Crystallin is made up of two polypeptides αA and αB encoded by the CRYAA gene on chromosome 21q22.3 and CRYAB gene on 11q22-q22.3, respectively. (bmj.com)
  • Alpha-crystallin A chain is a protein that in humans is encoded by the CRYAA gene. (wikipedia.org)
  • alpha-A crystallin (CRYAA), beta-B2 crystallin (CRYBB2), gamma-A crystallin (CRYGA), gamma-B crystallin (CRYGB), gamma-C crystallin (CRYGC), gamma-D crystallin (CRYGD), gap junction alpha-3 (GJA3), gap junction alpha-8 (GJA8), and PAX6 based on polymerase chain reaction and single strand conformation polymorphism (PCR-SSCP) analysis. (elsevier.com)
  • This gene, a beta acidic group member, encodes two proteins (crystallin, beta A3 and crystallin, beta A1) from a single mRNA, the latter protein is 17 aa shorter than crystallin, beta A3 and is generated by use of an alternate translation initiation site. (genecards.org)
  • 7 Although α- and βγ-crystallins are known to be the major structural components of the lens, these proteins are also expressed in other ocular and nonocular tissues. (arvojournals.org)
  • The function of βγ-crystallins is not well understood, though these proteins were recently associated with axonal regeneration of retinal ganglion cells (RGCs). (arvojournals.org)
  • Mouse eye extracts and recombinant proteins (Crystallin-alpha A and B) were resolved by electrophoresis, transferred to PVDF membrane and probed with anti-Crystallin alpha A (1:1000). (mybiosource.com)
  • Alpha-crystallin is the largest of the crystallins and is composed of 2 primary gene products--alpha-A and alpha-B. There are at least 5 different proteins comprising the beta-crystallins. (abcam.com)
  • Crystallins are the main structural proteins of the vertebrate eye lens, where they maintain the transparency and refractive index of the lens. (prospecbio.com)
  • Some of the proteins that were significantly upregulated included carbonic anhydrase 3 (5.3fold, p=0.0001), alpha crystallin B chain (3.3fold, p=0.0001) and beta crystallin B2 (2.99fold, p=0.00013). (arvojournals.org)
  • A method for expressing proteins as a fusion chimera with a domain of p26 or alpha crystallin type proteins to improve the protein stability and solubility when over expressed in bacteria such as E. coli is provided. (google.com)
  • Downregulated RV proteins included contractile elements: troponin T and C (-1.6 fold change), myosin regulatory light chain 2 (-1.9), cellular energetics modifier: fatty-acid binding protein (-1.5), and (3) ROS scavenger: superoxide dismutase 1 (-1.7). (stanford.edu)
  • In the RA, 22 proteins spots were altered including the following downregulated proteins contractile elements: tropomyosin 1 alpha chain (-1.9), cellular energetic proteins: ATP synthase (-1.5), fatty-acid binding protein (-2.5), and (3) polyubiquitin (-3.5). (stanford.edu)
  • β/γ-Crystallins are predominant structural proteins in the cytoplasm of lens fiber cells and share a similar fold composing of four Greek-key motifs divided into two domains. (bvsalud.org)
  • Only certain proteins are linked to proteopathy, possibly due to instability or other structural features of the monomeric protein that increase the probability of misconformation, [6] [8] which in nearly all instances involves an increase in beta-sheet secondary structure. (bionity.com)
  • UPS-mediated proteolysis includes 2 major steps: attachment of a chain of ubiquitin to the target protein molecule through a process known as ubiquitination and degradation of the ubiquitinated proteins by the 26S proteasome. (ahajournals.org)
  • Defects in CRYAB are the cause of myofibrillar alpha-B crystallin-related (MFM-CRYAB) [MIM:608810]. (abcam.com)
  • We report here that UPS proteolytic function is severely impaired in the heart of a mouse model of intrasarcoplasmic amyloidosis caused by cardiac-restricted expression of a human desmin-related myopathy-linked missense mutation of αB-crystallin (CryAB R120G ). (ahajournals.org)
  • A missense mutation (R120G) in the αB-crystallin (CryAB) gene encoding a molecular chaperone highly expressed in the heart has been shown to cause aberrant protein aggregation in the heart and result in desmin-related cardiomyopathies (DRC) in humans and transgenic (Tg) mice. (ahajournals.org)
  • 10,11 Mice carrying 3 copies of a Tg consisting of the mouse α-myosin heavy chain promoter and the CryAB R120G cDNA show no apparent cardiac abnormality at 1 month but develop concentric cardiac hypertrophy with diastolic malfunction at 3 months and die of CHF between 5 and 7 months. (ahajournals.org)
  • Numerous cataract-causing mutations have been identified in various β/γ-crystallins, but the mechanisms underlying cataract caused by most mutations remains uncharacterized. (bvsalud.org)
  • Anti-human Crystallin alpha A mAb, clone c9F2, is derived from hybridization of mouse SP2/O myeloma cells with spleen cells from BALB/c mice immunized with recombinant Crystallin alpha A. (mybiosource.com)
  • This antibody is not shown cross-activity about Crystallin alpha B. A : Crystallin-alpha A recombinant protein B : Crystallin-alpha B recombinant protein C : Mouse eye lysates The Cell lysates (5ug) were resolved by SDS-PAGE, transferred to PVDF membrane and probed with anti-human Crystallin alpha A antibody (1:3000). (mybiosource.com)
  • CRYBA4 Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 216 amino acids (1-196 a.a.) and having a molecular mass of 24.5kDa. (prospecbio.com)
  • Enzymatic assays indicated that recombinant polyserase-3 degrades the α-chain of fibrinogen as well as pro-urokinase-type plasminogen activator (pro-uPA). (biomedcentral.com)
  • Since lens central fiber cells lose their nuclei during development, these crystallins are made and then retained throughout life, making them extremely stab. (genecards.org)
  • Crystallins are the dominant structural components of the vertebrate eye lens. (genecards.org)
  • The role of the α-crystallins outside the lens is primarily attributed to chaperone and cell-protective functions. (arvojournals.org)
  • alpha-crystallins, a major protein of the ocular lens, are thought to play a role in maintaining lens transparency, which are composed of two gene products alpha-A and alpha-B, for acidic and basic, respectively. (mybiosource.com)
  • Purified alpha-A Crystallin from bovine lens. (abcam.com)
  • The mammalian lens contains 3 major classes of crystallins: alpha, beta, and gamma. (abcam.com)
  • The gamma-crystallins are monomeric, but there are at least 5 gamma crystallins identified in bovine and rat lens. (abcam.com)
  • Alpha-Crystallin comprises 40% of total lens protein composition. (abcam.com)
  • In addition to its structural role α-crystallin also functions as a molecular chaperone within the lens and other tissues. (bmj.com)
  • Beta-crystallin A4 (CRYBA4) is a member of the beta/gamma-crystallin family which are the dominant structural components of the vertebrate eye lens. (prospecbio.com)
  • CRYBB1 is a beta basic group member and undergoes extensive cleavage at its N-terminal extension during lens maturation. (prospecbio.com)
  • High concentrations of closely packed crystallins are required for lens transparency and the ability to focus light on the retina. (molvis.org)
  • Although the absence of alpha3 connexin had no obvious influence on the early stages of lens formation and the differentiation of lens fibers, mice homozygous for the disrupted alpha3 gene developed nuclear cataracts that were associated with the proteolysis of crystallins. (nih.gov)
  • Three main families of mammalian crystallins are α-, β-, and γ-crystallins. (arvojournals.org)
  • beta and gamma crystallins are also considered as a superfamily. (genecards.org)
  • β- and γ-crystallins share similar sequences, structure, and domain topology and have therefore been grouped together to form a superfamily of βγ-crystallins. (arvojournals.org)
  • The purpose of the study was to determine the effects of truncation of various regions of betaB1-crystallin on its structural properties and stability of heterooligomers formed by wild-type (WT) betaB1 or its deletion mutants with WT betaA3-crystallin. (isharonline.org)
  • PURPOSE: The purpose was to characterize the properties of a proteinase activity associated with betaA3-crystallin, which was isolated from the alpha-crystallin fraction of human lenses. (isharonline.org)
  • alpha-crystallins can be induced by heat shock and are members of the small heat shock protein (sHSP). (mybiosource.com)
  • Two additional function of alpha-crystallins are an autokinase activity and the participation in the intracellular architecture. (mybiosource.com)
  • Alpha crystallins are composed of two gene products: alpha-A and alpha-B, for acidic and basic, respectively. (mybiosource.com)
  • Alpha crystallins can be induced by heat shock and are members of the small heat shock protein (HSP20) family. (mybiosource.com)
  • The S228P mutation in βB1-crystallin has been linked to autosomal dominant congenital nuclear cataract. (bvsalud.org)
  • The CRYBA4 gene being a beta acidic group member is part of a gene cluster with beta-B1, beta-B2, and beta-B3. (prospecbio.com)
  • 2. The device of claim 1 , wherein the alpha-A-crystallin protein is a bovine alpha-A-crystallin protein. (google.com)
  • 16. The method of claim 12 , wherein the alpha-A-crystalline protein is a bovine alpha-A-crystallin protein. (google.com)
  • For 15 y, α B-crystallin (heat shock protein [Hsp] B5) has been labeled an autoantigen in multiple sclerosis (MS) based on humoral and cellular responses found in humans and animal models. (jimmunol.org)
  • The association of α B-crystallin (heat shock protein [Hsp] B5) with multiple sclerosis (MS) has been a puzzling story ( 1 ). (jimmunol.org)
  • Heat shock protein beta-8 (HspB8) (Alpha-crystallin C chain) (Smallstress protein-like protein HSP22) (E2-induced gene 1 protein)(Protein kinase H11). (nctu.edu.tw)
  • Mouse anti Human alpha B crystallin antibody recognizes alpha B crystallin, also known as heat shock protein beta-5, heat-shock 20 kD like-protein and renal carcinoma antigen NY-REN-27, rosenthal fiber component. (bio-rad-antibodies.com)
  • These light chains are nearly identical except for first 41 additional amino acid residues of the A1 light chain, containing repeated Ala-Pro sequence and two pairs of lysine residues located near the N -terminus [ 5 ]. (mdpi.com)
  • The highest logarithm of odds score (1.5) region 2q34-36.1, spanning the crystallin beta A2 ( CRYBA2 ) gene, showed no sequence changes. (molvis.org)
  • CRYBA1 (Crystallin Beta A1) is a Protein Coding gene. (genecards.org)
  • A missense mutation in alpha B-crystallin that changes proline 20 to an arginine leads to diminished anti-apoptotic activity compared with the native protein. (antibodies-online.com)
  • Immunofluorescence staining of phospho-alpha-B crystallin (Ser19) in U373 MG cells results in cytoplasmic staining. (abcam.com)
  • Alpha B-crystallin modulates cardiac hypertrophic response to mechanical overload. (usd.edu)
  • Beta-crystallins form aggregates of different sizes and are able to self-associate to form dimers or to form heterodimers with other beta-crystallins. (genecards.org)
  • The alpha-crystallin can be modified in vitro by carbamylation through a high-molecular-weight aggregates formation. (labome.org)
  • Seven protein regions exist in crystallins: four homologous motifs, a connecting peptide, and N- and C-terminal extensions. (genecards.org)
  • The β and γ crystallins are structurally related and consist of four similarly folded Greek key motifs organized into two domains. (molvis.org)
  • 0.5 ug/ml was sufficient for detection of 100 ng purified alphaA crystalline by colorimetric immunoblot analysis using Goat Anti-Mouse IgG:HRP as the secondary.Alpha A Crystallin Antibody.Use in Immunoblotting reported in scientific literature (PMID 28546921). (novusbio.com)
  • Western blot analysis of whole cell lysates probed with alpha B crystallin antibody followed by detection with HRP conjugated Goat anti Mouse IgG (1/10,000, STAR207P ) and visualized on the ChemiDoc MP with 4 second exposure. (bio-rad-antibodies.com)
  • Baba, Oshitari, Yamamoto: Level of vitreous alpha-B crystallin in eyes with rhegmatogenous retinal detachment. (antibodies-online.com)
  • The S228P mutation modified the refolding pathway of βB1-crystallin by affecting the formation of the dimeric intermediate but not the monomeric intermediate. (bvsalud.org)
  • Zeta-crystallin with NADP-dependent quinone reductase activity (QOR). (umbc.edu)
  • We have previously described the identification and characterization of polyserase-1 and polyserase-2, two human serine proteases containing three different catalytic domains within the same polypeptide chain. (biomedcentral.com)
  • Following our interest in the study of the human degradome, we have cloned a human liver cDNA encoding polyserase-3, a new protease with tandem serine protease domains in the same polypeptide chain. (biomedcentral.com)
  • Moreover, analysis of post-translational mechanisms operating in polyserase-3 maturation showed that its two protease domains remain as integral parts of the same polypeptide chain. (biomedcentral.com)
  • Structural and evolutionary relationships among five members of the human gamma-crystallin gene family. (expasy.org)
  • This study clearly demonstrated that both the length and electrostatic charge of the COOH-terminal segment play crucial roles in governing the structural stability and chaperone activity of alphaB-crystallin . (antibodies-online.com)
  • Yan H, Yao L, Hui Y. [Decreased chaperone activity of alpha-crystallin by carbamylation in vitro]. (labome.org)
  • genome codes for An organic compound made of amino acids arranged in a linear chain, joined together by peptide bonds between the carboxyl and amino groups of the adjacent amino acid residues. (godandscience.org)