Genomes and Genes
Gene Symbol: alpha B-crystallin
Description: crystallin alpha B
Alias: CMD1II, CRYA2, CTPP2, CTRCT16, HEL-S-101, HSPB5, MFM2, alpha-crystallin B chain, epididymis secretory protein Li 101, heat shock protein beta-5, heat-shock 20 kD like-protein, renal carcinoma antigen NY-REN-27, rosenthal fiber component
Products: alpha B-crystallin
- Hanson S, Hasan A, Smith D, Smith J. The major in vivo modifications of the human water-insoluble lens crystallins are disulfide bonds, deamidation, methionine oxidation and backbone cleavage. Exp Eye Res. 2000;71:195-207 pubmed
- Somasundaram T, Bhat S. Developmentally dictated expression of heat shock factors: exclusive expression of HSF4 in the postnatal lens and its specific interaction with alphaB-crystallin heat shock promoter. J Biol Chem. 2004;279:44497-503 pubmed
- Berry V, Francis P, Reddy M, Collyer D, Vithana E, Mackay I, et al. Alpha-B crystallin gene (CRYAB) mutation causes dominant congenital posterior polar cataract in humans. Am J Hum Genet. 2001;69:1141-5 pubmed..The mutation resulted in a frameshift in codon 150 and produced an aberrant protein consisting of 184 residues. This is the first report of a mutation, in this gene, resulting in isolated congenital cataract. ..
- Chen Q, Ma J, Yan M, Mothobi M, Liu Y, Zheng F. A novel mutation in CRYAB associated with autosomal dominant congenital nuclear cataract in a Chinese family. Mol Vis. 2009;15:1359-65 pubmed..These results supported the idea that the novel R11H mutation was responsible for the autosomal dominant nuclear congenital cataract in this pedigree. ..
- Ghosh J, Shenoy A, Clark J. Interactions between important regulatory proteins and human alphaB crystallin. Biochemistry. 2007;46:6308-17 pubmed..This is the first report in which sequences involved in interactions with regulatory proteins, including FGF-2, NGF-beta, VEGF, insulin, and beta-catenin, were identified in a small heat shock protein. ..
- Lin D, Barbash O, Kumar K, Weber J, Harper J, Klein Szanto A, et al. Phosphorylation-dependent ubiquitination of cyclin D1 by the SCF(FBX4-alphaB crystallin) complex. Mol Cell. 2006;24:355-66 pubmed..We conclude that SCF(FBX4-alphaB crystallin) is an E3 ubiquitin ligase that promotes ubiquitin-dependent degradation of Thr286-phosphorylated cyclin D1. ..
- MacCoss M, McDonald W, Saraf A, Sadygov R, Clark J, Tasto J, et al. Shotgun identification of protein modifications from protein complexes and lens tissue. Proc Natl Acad Sci U S A. 2002;99:7900-5 pubmed..The method presented will be useful in discovering co- and posttranslational modifications of proteins. ..
- Rajaraman K, Raman B, Ramakrishna T, Rao C. Interaction of human recombinant alphaA- and alphaB-crystallins with early and late unfolding intermediates of citrate synthase on its thermal denaturation. FEBS Lett. 2001;497:118-23 pubmed
- Miesbauer L, Zhou X, Yang Z, Sun Y, Smith D, Smith J. Post-translational modifications of water-soluble human lens crystallins from young adults. J Biol Chem. 1994;269:12494-502 pubmed..This mass spectrometric characterization of the total water-soluble proteins from normal young adult lenses provides a reference data base for future investigations of the modifications present in aged and cataractous lenses. ..
- Ousman S, Tomooka B, van Noort J, Wawrousek E, O Connor K, Hafler D, et al. Protective and therapeutic role for alphaB-crystallin in autoimmune demyelination. Nature. 2007;448:474-9 pubmed..Thus, the immune response against a negative regulator of inflammation, CRYAB, in multiple sclerosis, would exacerbate inflammation and demyelination. This can be countered by giving CRYAB itself for therapy of ongoing disease. ..
- Michiel M, Skouri Panet F, Duprat E, Simon S, Férard C, Tardieu A, et al. Abnormal assemblies and subunit exchange of alphaB-crystallin R120 mutants could be associated with destabilization of the dimeric substructure. Biochemistry. 2009;48:442-53 pubmed publisher..The whole of the results demonstrated the importance of the R120 residue for structural integrity, both static and dynamic, in relation with function. ..
- Tang Q, Liu Y, Zhu X, Li Y, Zhu J, Zhang J, et al. Expression and prognostic significance of the alpha B-crystallin gene in human hepatocellular carcinoma. Hum Pathol. 2009;40:300-5 pubmed publisher..It may also be involved in the malignant transformation of hepatocytes. ..
- Gupta R, Srivastava O. Deamidation affects structural and functional properties of human alphaA-crystallin and its oligomerization with alphaB-crystallin. J Biol Chem. 2004;279:44258-69 pubmed
- Mao Y, Zhang D, Lin H, Xiong L, Liu Y, Li Q, et al. Alpha B-crystallin is a new prognostic marker for laryngeal squamous cell carcinoma. J Exp Clin Cancer Res. 2012;31:101 pubmed publisher..027) and lymphatic metastasis (P = 0.015) were independent prognosis factors for LSCC. The data suggest that ?B-crystallin expression is correlated with malignant phenotypes of LSCC and it may serve as a novel prognostic factor for LSCC. ..
- Peschek J, Braun N, Franzmann T, Georgalis Y, Haslbeck M, Weinkauf S, et al. The eye lens chaperone alpha-crystallin forms defined globular assemblies. Proc Natl Acad Sci U S A. 2009;106:13272-7 pubmed publisher..This propensity might explain the previously reported polydisperse nature of alpha-crystallin. ..
- Duncan B, Zhao K. HMGA1 mediates the activation of the CRYAB promoter by BRG1. DNA Cell Biol. 2007;26:745-52 pubmed..Our data indicate that HMGA1 nonhistone chromatin proteins, the SWI/SNF chromatin remodeling complexes, and sequence-specific transcription factors act together to regulate the expression of the CRYAB gene. ..
- Raman B, Ban T, Sakai M, Pasta S, Ramakrishna T, Naiki H, et al. AlphaB-crystallin, a small heat-shock protein, prevents the amyloid fibril growth of an amyloid beta-peptide and beta2-microglobulin. Biochem J. 2005;392:573-81 pubmed..Our study sheds light on the role of small heat-shock proteins in protein conformational diseases, particularly in Alzheimer's disease. ..
- Vos M, Kanon B, Kampinga H. HSPB7 is a SC35 speckle resident small heat shock protein. Biochim Biophys Acta. 2009;1793:1343-53 pubmed publisher..Unlike HSPB1 and HSPB5, that chaperoned heat unfolded substrates and kept them folding competent, HSPB7 did not support refolding...
- Safieh L, Khan A, Alkuraya F. Identification of a novel CRYAB mutation associated with autosomal recessive juvenile cataract in a Saudi family. Mol Vis. 2009;15:980-4 pubmed..This is the first report of a recessive mutation in CRYAB causing cataract. Based on recent knowledge of the structure and function of this small heat shock protein, we speculate on the potential mutational mechanism. ..
- Jehle S, Rajagopal P, Bardiaux B, Markovic S, Kühne R, Stout J, et al. Solid-state NMR and SAXS studies provide a structural basis for the activation of alphaB-crystallin oligomers. Nat Struct Mol Biol. 2010;17:1037-42 pubmed publisher..Intermolecular restraints from NMR and volumetric restraints from SAXS were combined to calculate a model of a 24-subunit alphaB oligomer with tetrahedral symmetry. ..
- Rajasekaran N, Connell P, Christians E, Yan L, Taylor R, Orosz A, et al. Human alpha B-crystallin mutation causes oxido-reductive stress and protein aggregation cardiomyopathy in mice. Cell. 2007;130:427-39 pubmed..These findings demonstrate that dysregulation of G6PD activity is necessary and sufficient for maladaptive reductive stress and suggest a novel therapeutic target for abrogating R120GCryAB cardiomyopathy and heart failure in humans. ..
- Ghosh J, Houck S, Clark J. Interactive domains in the molecular chaperone human alphaB crystallin modulate microtubule assembly and disassembly. PLoS ONE. 2007;2:e498 pubmed..These are the first experimental results in support of the functional importance of the dynamic subunit model of small heat shock proteins. ..
- Ghosh J, Estrada M, Clark J. Interactive domains for chaperone activity in the small heat shock protein, human alphaB crystallin. Biochemistry. 2005;44:14854-69 pubmed..The results suggested that interactive sequences in human alphaB crystallin have dual roles in subunit-subunit assembly and chaperone activity. ..
- Ecroyd H, Carver J. Crystallin proteins and amyloid fibrils. Cell Mol Life Sci. 2009;66:62-81 pubmed publisher..Finally, the fibril-forming propensity of the crystallin proteins and its implications for cataract formation are described along with the potential use of fibrillar crystallin proteins as bionanomaterials. ..
- Swaim C, Smith D, Smith J. The reaction of alpha-crystallin with the cross-linker 3,3'-dithiobis(sulfosuccinimidyl propionate) demonstrates close proximity of the C termini of alphaA and alphaB in the native assembly. Protein Sci. 2004;13:2832-5 pubmed..This cross-link provides experimental evidence supporting theoretical structural models that place the C termini of alphaA and alphaB within close proximity in the native aggregate. ..
- Kamradt M, Chen F, Sam S, Cryns V. The small heat shock protein alpha B-crystallin negatively regulates apoptosis during myogenic differentiation by inhibiting caspase-3 activation. J Biol Chem. 2002;277:38731-6 pubmed..Taken together, our findings indicate that alpha B-crystallin is a novel negative regulator of myogenic apoptosis that directly links the differentiation program to apoptosis resistance. ..
- Thedieck C, Kalbacher H, Kratzer U, Lammers R, Stevanovic S, Klein G. alpha B-crystallin is a cytoplasmic interaction partner of the kidney-specific cadherin-16. J Mol Biol. 2008;378:145-53 pubmed publisher..These findings suggest that the interaction of Ksp-cadherin with alpha B-crystallin is important for the connection of Ksp-cadherin to the cytoskeleton and thus for maintaining tissue integrity in the kidney. ..
- Dimberg A, Rylova S, Dieterich L, Olsson A, Schiller P, Wikner C, et al. alphaB-crystallin promotes tumor angiogenesis by increasing vascular survival during tube morphogenesis. Blood. 2008;111:2015-23 pubmed..Hereby we identify the small heat shock protein family as a novel class of angiogenic modulators. ..
- Raju M, Santhoshkumar P, Sharma K. Cataract-causing ?AG98R-crystallin mutant dissociates into monomers having chaperone activity. Mol Vis. 2011;17:7-15 pubmed..The chaperone activity of the dissociated subunits against four client proteins suggests that the ?A-crystallin subunits are the minimal units of chaperone activity. ..
- Sun W, Xiao X, Li S, Guo X, Zhang Q. Mutation analysis of 12 genes in Chinese families with congenital cataracts. Mol Vis. 2011;17:2197-206 pubmed..intronic regions of 12 genes were analyzed by cycle sequencing, including the alpha A crystallin (CRYAA), alpha B crystallin (CRYAB), beta A1 crystallin (CRYBA1), beta A4 crystallin (CRYBA4), beta B1 crystallin (CRYBB1), beta B2 ..
- Treweek T, Ecroyd H, Williams D, Meehan S, Carver J, Walker M. Site-directed mutations in the C-terminal extension of human alphaB-crystallin affect chaperone function and block amyloid fibril formation. PLoS ONE. 2007;2:e1046 pubmed..The capacity to genetically modify alphaB-crystallin for improved ability to block amyloid fibril formation provides a platform for the future use of such engineered molecules in treatment of diseases caused by amyloid fibril formation. ..
- Jehle S, van Rossum B, Stout J, Noguchi S, Falber K, Rehbein K, et al. alphaB-crystallin: a hybrid solid-state/solution-state NMR investigation reveals structural aspects of the heterogeneous oligomer. J Mol Biol. 2009;385:1481-97 pubmed publisher..8. This steep pH-dependent switch may be important for alphaB to function optimally (e.g., to preserve the filament integrity of cardiac muscle proteins such as actin and desmin during cardiac ischemia, which is accompanied by acidosis). ..
- Agarwal T, Khokhar S, Kumar M, Kaur P, Roy T, Dada R. Mutation screening and genotype phenotype correlation of ?-crystallin, ?-crystallin and GJA8 gene in congenital cataract. Mol Vis. 2011;17:693-707 pubmed..L281C) in GJA8 may lead to lens opacification resulting in cataract formation. This study further expands the mutation spectrum of congenital cataract and help understanding how mutant proteins lead to opacification of lens. ..
- Mao Y, Liu J, Xiang H, Li D. Human alphaA- and alphaB-crystallins bind to Bax and Bcl-X(S) to sequester their translocation during staurosporine-induced apoptosis. Cell Death Differ. 2004;11:512-26 pubmed..Thus, our results demonstrate a novel antiapoptotic mechanism for alpha-crystallins. ..
- Noh S, Jeong W, Rho J, Shin D, Ahn H, Park W, et al. Sensitization of RPE cells by alphaB-crystallin siRNA to SAHA-induced stage 1 apoptosis through abolishing the association of alphaB-crystallin with HDAC1 in SC35 speckles. Invest Ophthalmol Vis Sci. 2008;49:4753-9 pubmed publisher..Knockout of alphaB-crystallin may be a promising new approach to enhance therapeutic potency for proliferative vitreoretinopathy without compromising efficacy. ..
- Numoto N, Kita A, Fujii N, Miki K. A P39R mutation at the N-terminal domain of human ?B-crystallin regulates its oligomeric state and chaperone-like activity. Biochem Biophys Res Commun. 2012;425:601-6 pubmed publisher..The Pro residue at the N-terminal domain of ?B-crystallin is important for oligomerization and function. ..
- Iwaki T, Kume Iwaki A, Goldman J. Cellular distribution of alpha B-crystallin in non-lenticular tissues. J Histochem Cytochem. 1990;38:31-9 pubmed..A close correlation with markers of oxidative activity suggests that alpha B-crystallin is expressed in cells that have high levels of oxidative function. ..
- Li H, Li C, Lu Q, Su T, Ke T, Li D, et al. Cataract mutation P20S of alphaB-crystallin impairs chaperone activity of alphaA-crystallin and induces apoptosis of human lens epithelial cells. Biochim Biophys Acta. 2008;1782:303-9 pubmed publisher..These results suggest that the changes in the structure and function of the alpha-crystallin complex and cytotoxicity are vital factors in the pathogenesis of congenital cataract linked to the P20S mutation in the alphaB-crystallin. ..
- Murugesan R, Santhoshkumar P, Sharma K. Role of alphaBI5 and alphaBT162 residues in subunit interaction during oligomerization of alphaB-crystallin. Mol Vis. 2008;14:1835-44 pubmed
- Ghosh J, Houck S, Clark J. Interactive sequences in the stress protein and molecular chaperone human alphaB crystallin recognize and modulate the assembly of filaments. Int J Biochem Cell Biol. 2007;39:1804-15 pubmed publisher..The results support the hypothesis that interactive domains in alphaB crystallin have multiple functions in stabilizing the cytoskeleton and protecting cytosolic proteins from unfolding...
- Jeong W, Rho J, Yoon Y, Yoo S, Jeong N, Ryu W, et al. Cytoplasmic and nuclear anti-apoptotic roles of ?B-crystallin in retinal pigment epithelial cells. PLoS ONE. 2012;7:e45754 pubmed publisher..Taken together, these results suggest that ?B-crystallin prevents caspase activation by physically interacting with caspase subtypes in the cytoplasm and nucleus, thereby protecting RPE cells from MGO-induced apoptosis. ..
- Boelens W, Croes Y, de Jong W. Interaction between alphaB-crystallin and the human 20S proteasomal subunit C8/alpha7. Biochim Biophys Acta. 2001;1544:311-9 pubmed
- Spinozzi F, Mariani P, Rustichelli F, Amenitsch H, Bennardini F, Mura G, et al. Temperature dependence of chaperone-like activity and oligomeric state of alphaB-crystallin. Biochim Biophys Acta. 2006;1764:677-87 pubmed..In particular, the changes in the inner radius as well as the thickness and dispersion of the protein shell, account for the preservation of the chaperone-like activity. ..
- Kumar M, Kapoor M, Sinha S, Reddy G. Insights into hydrophobicity and the chaperone-like function of alphaA- and alphaB-crystallins: an isothermal titration calorimetric study. J Biol Chem. 2005;280:21726-30 pubmed..Our results indicate that although hydrophobicity appears to be a factor in determining the chaperone-like activity of alpha-crystallins, it does not quantitatively correlate with the chaperone function of alpha-crystallins. ..
- Derham B, Harding J. Alpha-crystallin as a molecular chaperone. Prog Retin Eye Res. 1999;18:463-509 pubmed..Increased expression of alphaB-crystallin has been seen in many pathological states. Conformational disorders, including cataract may have a common aetiology and potentially a common therapy. ..
- Liu L, Ghosh J, Clark J, Jiang S. Studies of alphaB crystallin subunit dynamics by surface plasmon resonance. Anal Biochem. 2006;350:186-95 pubmed
- Ghosh J, Houck S, Clark J. Interactive sequences in the molecular chaperone, human alphaB crystallin modulate the fibrillation of amyloidogenic proteins. Int J Biochem Cell Biol. 2008;40:954-67 pubmed..This is the first characterization of specific bioactive peptides synthesized on the basis of interactive domains in the small heat shock protein, alphaB crystallin that protect against the fibrillation of amyloidogenic proteins. ..
- Moyano J, Evans J, Chen F, Lu M, Werner M, Yehiely F, et al. AlphaB-crystallin is a novel oncoprotein that predicts poor clinical outcome in breast cancer. J Clin Invest. 2006;116:261-70 pubmed..Our data also implicate the MEK/ERK pathway as a potential therapeutic target for these tumors. ..
- Sreelakshmi Y, Sharma K. Recognition sequence 2 (residues 60-71) plays a role in oligomerization and exchange dynamics of alphaB-crystallin. Biochemistry. 2005;44:12245-52 pubmed..Our results establish the importance of residues 60-71 in oligomerization of alphaB-crystallin and subunit interaction between alphaB- and alphaA-crystallins. ..
- van den IJssel P, Wheelock R, Prescott A, Russell P, Quinlan R. Nuclear speckle localisation of the small heat shock protein alpha B-crystallin and its inhibition by the R120G cardiomyopathy-linked mutation. Exp Cell Res. 2003;287:249-61 pubmed
- Bagneris C, Bateman O, Naylor C, Cronin N, Boelens W, Keep N, et al. Crystal structures of alpha-crystallin domain dimers of alphaB-crystallin and Hsp20. J Mol Biol. 2009;392:1242-52 pubmed publisher..The empty pockets and groove provide a starting model for designing drugs to inhibit those sHsps that have a negative effect on cancer treatment. ..
- Aquilina J, Shrestha S, Morris A, Ecroyd H. Structural and functional aspects of hetero-oligomers formed by the small heat shock proteins ?B-crystallin and HSP27. J Biol Chem. 2013;288:13602-9 pubmed publisher..This represents the first quantitative evaluation of ?B/HSP27 subunit exchange, and the results are discussed in the broader context of regulation of function and cellular proteostasis. ..
- Boslooper K, King Yin Lam A, Gao J, Weinstein S, Johnson N. The clinicopathological roles of alpha-B-crystallin and p53 expression in patients with head and neck squamous cell carcinoma. Pathology. 2008;40:500-4 pubmed publisher..032). The expression of p53 and alpha-B-crystallin were related to the differentiation and site of the HNSCC. Alpha-B-crystallin was not a prognostic marker for HNSCC. ..
- Ghosh J, Shenoy A, Clark J. N- and C-Terminal motifs in human alphaB crystallin play an important role in the recognition, selection, and solubilization of substrates. Biochemistry. 2006;45:13847-54 pubmed..The results presented in this report established that interactive domains in the N- and C-termini of human alphaB crystallin are important for the recognition, selection, and solubility of unfolding substrate proteins. ..
- Ghosh J, Estrada M, Houck S, Clark J. The function of the beta3 interactive domain in the small heat shock protein and molecular chaperone, human alphaB crystallin. Cell Stress Chaperones. 2006;11:187-97 pubmed..The results characterized the importance of the exposed side chains of Glu-78, Lys-82, and His-83 in the interactive beta3 sequence of the alpha crystallin core domain in alphaB crystallin for chaperone function. ..
- den Engelsman J, Keijsers V, de Jong W, Boelens W. The small heat-shock protein alpha B-crystallin promotes FBX4-dependent ubiquitination. J Biol Chem. 2003;278:4699-704 pubmed
- Inagaki N, Hayashi T, Arimura T, Koga Y, Takahashi M, Shibata H, et al. Alpha B-crystallin mutation in dilated cardiomyopathy. Biochem Biophys Res Commun. 2006;342:379-86 pubmed
- Chávez Zobel A, Loranger A, Marceau N, Theriault J, Lambert H, Landry J. Distinct chaperone mechanisms can delay the formation of aggresomes by the myopathy-causing R120G alphaB-crystallin mutant. Hum Mol Genet. 2003;12:1609-20 pubmed
- Saha S, Das K. Relationship between chaperone activity and oligomeric size of recombinant human alphaA- and alphaB-crystallin: a tryptic digestion study. Proteins. 2004;57:610-7 pubmed..The results also suggest that the presence of the alpha-crystallin domain and hydrophobic clefts on the protein surface, which correlate poorly with oligomeric size, are important for chaperone function...
- Hayes V, Devlin G, Quinlan R. Truncation of alphaB-crystallin by the myopathy-causing Q151X mutation significantly destabilizes the protein leading to aggregate formation in transfected cells. J Biol Chem. 2008;283:10500-12 pubmed publisher..In support of this hypothesis, we show that Q151X alphaB-crystallin is found mainly in the insoluble fraction of cell extracts from transient transfected cells, due to the formation of cytoplasmic aggregates. ..
- Rekas A, Jankova L, Thorn D, Cappai R, Carver J. Monitoring the prevention of amyloid fibril formation by alpha-crystallin. Temperature dependence and the nature of the aggregating species. FEBS J. 2007;274:6290-304 pubmed..It was shown that alphaB-crystallin interrupted alpha-synuclein aggregation at its earliest stages, most likely by binding to partially folded monomers and thereby preventing their aggregation into fibrillar structures. ..
- Narayanan S, Kamps B, Boelens W, Reif B. alphaB-crystallin competes with Alzheimer's disease beta-amyloid peptide for peptide-peptide interactions and induces oxidation of Abeta-Met35. FEBS Lett. 2006;580:5941-6 pubmed..J. et al. (1999) The molecular chaperone alphaB-crystallin enhances amyloid-beta neurotoxicity. Biochem. Biophys. Res. Commun. 262, 152-156.]. ..
- Vicart P, Caron A, Guicheney P, Li Z, Prevost M, Faure A, et al. A missense mutation in the alphaB-crystallin chaperone gene causes a desmin-related myopathy. Nat Genet. 1998;20:92-5 pubmed..These results are the first to identify a defect in a molecular chaperone as a cause for an inherited human muscle disorder. ..
- den Engelsman J, Bennink E, Doerwald L, Onnekink C, Wunderink L, Andley U, et al. Mimicking phosphorylation of the small heat-shock protein alphaB-crystallin recruits the F-box protein FBX4 to nuclear SC35 speckles. Eur J Biochem. 2004;271:4195-203 pubmed
- Huang Z, Cheng Y, Chiu P, Cheung F, Nicholls J, Kwong D, et al. Tumor suppressor Alpha B-crystallin (CRYAB) associates with the cadherin/catenin adherens junction and impairs NPC progression-associated properties. Oncogene. 2012;31:3709-20 pubmed publisher..Taken together, these results indicate that CRYAB functions to suppress NPC progression by associating with the cadherin/catenin adherens junction and modulating the β-catenin function. ..
- Dubin R, Ally A, Chung S, Piatigorsky J. Human alpha B-crystallin gene and preferential promoter function in lens. Genomics. 1990;7:594-601 pubmed
- Fu L, Liang J. Detection of protein-protein interactions among lens crystallins in a mammalian two-hybrid system assay. J Biol Chem. 2002;277:4255-60 pubmed..These results show that the two-hybrid system can detect interactions among various crystallins and may be used in mapping interaction domains. ..
- Pasta S, Raman B, Ramakrishna T, Rao C. Role of the conserved SRLFDQFFG region of alpha-crystallin, a small heat shock protein. Effect on oligomeric size, subunit exchange, and chaperone-like activity. J Biol Chem. 2003;278:51159-66 pubmed..Thus, our study shows that the SRLFDQFFG sequence is one of the critical motifs in structure-function regulation of alpha A- and alpha B-crystallin. ..
- Sacconi S, Feasson L, Antoine J, Pecheux C, Bernard R, Cobo A, et al. A novel CRYAB mutation resulting in multisystemic disease. Neuromuscul Disord. 2012;22:66-72 pubmed publisher..We propose that impairment of alpha-B crystallin dimerization may also be relevant to the pathogenesis of these disorders. ..
- Selcen D, Engel A. Myofibrillar myopathy caused by novel dominant negative alpha B-crystallin mutations. Ann Neurol. 2003;54:804-10 pubmed
- Zhang H, Rajasekaran N, Orosz A, Xiao X, Rechsteiner M, Benjamin I. Selective degradation of aggregate-prone CryAB mutants by HSPB1 is mediated by ubiquitin-proteasome pathways. J Mol Cell Cardiol. 2010;49:918-30 pubmed publisher..We conclude that such selective interactions between CryAB mutants and HSPB1 overexpression might have important implications for the clinical manifestations and potential treatment. ..
- Liu M, Ke T, Wang Z, Yang Q, Chang W, Jiang F, et al. Identification of a CRYAB mutation associated with autosomal dominant posterior polar cataract in a Chinese family. Invest Ophthalmol Vis Sci. 2006;47:3461-6 pubmed..This study identified a second novel mutation in CRYAB in a large Chinese cataract family. Together, these results provide strong evidence that CRYAB is a pathogenic gene for congenital cataract. ..
- Ghosh J, Estrada M, Clark J. Structure-based analysis of the beta8 interactive sequence of human alphaB crystallin. Biochemistry. 2006;45:9878-86 pubmed..The results established the beta3-beta8-beta9 surface of the alpha crystallin core domain as an interface for complex assembly and chaperone activity. ..
- Kamradt M, Lu M, Werner M, Kwan T, Chen F, Strohecker A, et al. The small heat shock protein alpha B-crystallin is a novel inhibitor of TRAIL-induced apoptosis that suppresses the activation of caspase-3. J Biol Chem. 2005;280:11059-66 pubmed..Moreover, these results demonstrate that targeted inhibition of alpha B-crystallin promotes TRAIL-induced apoptosis, thereby suggesting a novel strategy to overcome TRAIL resistance in cancer. ..
- Stegh A, Kesari S, Mahoney J, Jenq H, Forloney K, Protopopov A, et al. Bcl2L12-mediated inhibition of effector caspase-3 and caspase-7 via distinct mechanisms in glioblastoma. Proc Natl Acad Sci U S A. 2008;105:10703-8 pubmed publisher..revealed a striking induction of the small heat shock protein alpha-basic-crystallin (alphaB-crystallin/HspB5), a link reinforced by robust alphaB-crystallin expression in Bcl2L12-expressing orthotopic glioma and strong ..