Conserved Protein Domain Family
PRKCSH

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pfam07915: PRKCSH 
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Glucosidase II beta subunit-like protein
The sequences found in this family are similar to a region found in the beta-subunit of glucosidase II, which is also known as protein kinase C substrate 80K-H (PRKCSH). The enzyme catalyzes the sequential removal of two alpha-1,3-linked glucose residues in the second step of N-linked oligosaccharide processing. The beta subunit is required for the solubility and stability of the heterodimeric enzyme, and is involved in retaining the enzyme within the endoplasmic reticulum. Mutations in the gene coding for PRKCSH have been found to be involved in the development of autosomal dominant polycystic liver disease (ADPLD), but the precise role the protein has in the pathogenesis of this disease is unknown. This family also includes an ER sensor for misfolded glycoproteins and is therefore likely to be a generic sugar binding domain.
Statistics
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PSSM-Id: 311734
View PSSM: pfam07915
Aligned: 100 rows
Threshold Bit Score: 29.0595
Threshold Setting Gi: 74709301
Created: 22-Jul-2016
Updated: 4-Aug-2016
Structure
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Program:
Drawing:
Aligned Rows:
PubMed ReferencesClick to see Conserved Features Help

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
3AIH_A         3 APCL-LKTKDWWTYEFCY----GRHIQQYHMed--seIKGEVLYLG-YYqs-------aFDWDDETak------------ 55
gi 74644875  537 SYCL-DDILDNYVYTICF----QRPMTEGVIyqaedkVDGKKVLIG-RFktsgfnvdlnMEKYAEHlkatydeksdlisn 610
gi 74605164  502 DFCV-DSELNSYIYRLCYsndkGGDIVQIENk-----PDGNKVVIG-RYrgft--idtnSKYDVYLqslkyrypntevgn 572
gi 74691767  505 DFCI-SSKFDEYTYHICF----NPDTGRILQvq---dRDRSVVLVG-HFkdfk---lvsQRARDAYinqlqtratdvdli 572
gi 74709301   30 GNCW-LRQA---KNGRCQ----VLYKTELSKe-----ECCSTGRLStSWte----------------------------- 67
gi 23821549   34 GVCWlQQGKE----ATCS----LVLKTQVSRe-----ECCASGNINtAWs------------------------------ 70
gi 82087463   24 GNCW--YEQG--KNGRCQ----MLYLSEMSRe-----DCCRSERLGmAWte----------------------------- 61
gi 123891431  30 GNCW-LQQSK---NGRCR------ELLKMNVsq---qECCRSGRLGsay------------------------------- 65
gi 205591064  33 GNCW-LQQGK---NGRCQ----VLYMPGMSRe-----ECCRSGRLGtSWte----------------------------- 70
gi 122102926 212 GTCW-QTHLGs---GKCG----QVFSTDISRs----eCCGSSQSFSyt-------------------------------- 247
                         90       100       110
                 ....*....|....*....|....*....|....*..
3AIH_A        56 ---------------asKQHRLKrYHSQTYGNGSK-C 76
gi 74644875  611 laaiqdddgnmqhyvfgNLNELNnGLVLEYENGDQ-C 646
gi 74605164  573 hlssdtveigkeeillgNLPDLNnGLILEYRGGTK-C 608
gi 74691767  573 ghlsnyspgaghdlllgNLPDIHnGLLLSYGNGHK-C 608
gi 74709301   68 ----------------eDVNDNTlFKWMIFNGGAPnC 88
gi 23821549   71 ----------------nFTHPGNkISLLGFLGLVH-C 90
gi 82087463   62 ----------------eDVPSNTlFRWLLMHGGAPnC 82
gi 123891431  66 --------------tgeQVSTATlFRWMAFSGGAPnC 88
gi 205591064  71 ----------------eDVPNSTlFRWMIFNGGAPnC 91
gi 122102926 248 ---------------drELSSVEyFFATAIGGGVE-C 268
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