Conserved Protein Domain Family
ACD_alphaB-crystallin_HspB5

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cd06498: ACD_alphaB-crystallin_HspB5 
Alpha-crystallin domain found in the small heat shock protein (sHsp) alphaB-crystallin (HspB5, 20kDa). sHsps are molecular chaperones that suppress protein aggregation and protect against cell stress, and are generally active as large oligomers consisting of multiple subunits. Alpha crystallin, an abundant protein in the mammalian lens, is a large (700 kDa) heteropolymer composed of HspB4 and HspB5, generally in a molar ratio of HspB4:HspB5 of 3:1. HspB4 does not belong to this group. HspB5 shows increased synthesis in response to stress. HspB5 is also expressed constitutively in other tissues including brain, heart, and type I and type IIa skeletal muscle fibers, and in several cancers including gliomas, renal cell carcinomas, basal-like and metaplastic breast carcinomas, and head and neck cancer. Its functions include effects on the apoptotic pathway and on metastasis. Phosphorylation of HspB5 reduces its oligomerization and anti-apoptotic activities. HspB5 is protective in demyelinating disease such as multiple sclerosis (MS), being a negative regulator of inflammation. In early active MS lesions it is the most abundant gene transcript and an autoantigen, the immune response against it would disrupt its function and worsen inflammation and demyelination. Given as therapy for ongoing demyelinating disease it may counteract this effect. It is an autoantigen in the pathogenesis of various other inflammatory disorders including Lens-associated uveitis (LAU), and Behcet's disease. Mutations in HspB5 have been associated with diseases including dominant cataract and desmin-related myopathy.
Statistics
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PSSM-Id: 107246
View PSSM: cd06498
Aligned: 3 rows
Threshold Bit Score: 172.894
Threshold Setting Gi: 6166128
Created: 18-Mar-2008
Updated: 17-Jan-2013
Structure
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Aligned Rows:
 
putative dimer
Feature 1:putative dimer interface [polypeptide binding site]
Evidence:

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
Feature 1      #####     # # ##                                    #                    ##    
P02511     67 EMRLEKdRFSVNLDVKHFSPEELKVKVLgDVIEVHGKHeeRQDeHGFISREFHRKYRIPADVDPltITSSLSsDGVLTVN 146 human
Q91312     65 EMRLEKdKFSINLDVKHFSPEELKVKVSgDFIEIHGKHeeRQDeHGYVSRDFQRRYKIPVDVDPlsITSSLSpDGVLTVC 144 bullfrog
Q05713     66 EMRLEKdKFSVNLDVKHFSPEELKVKVLgDMIEIHGKHeeRQDeHGFIAREFSRKYRIPADVDPltITSSLSlDGVLTVS 145 chicken
Feature 1         
P02511    147 GPRK 150 human
Q91312    145 GPRK 148 bullfrog
Q05713    146 APRK 149 chicken

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