3AIH


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: 336863
View PSSM: pfam07915
Aligned: 101 rows
Threshold Bit Score: 30.5918
Threshold Setting Gi: 74605164
Created: 20-Mar-2017
Updated: 23-May-2017
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         5 CL-LKTKDWWTYEFCY----GRHIQQYHmEds---eIKGEVLYLG-YYq---------SAfdWDDETak----------- 55
gi 74644875  539 CL-DDILDNYVYTICF----QRPMTEGV-IyqaedkVDGKKVLIG-RFktsgfnvdlnME--KYAEHlkatydeksdlis 609
gi 74605164  504 CV-DSELNSYIYRLCYsndkGGDIVQIE-Nk-----PDGNKVVIG-RYrgft--idtnSK--YDVYLqslkyrypntevg 571
gi 74691767  507 CI-SSKFDEYTYHICF----NPDTGRIL-Qvq---dRDRSVVLVG-HFkdfk---lvsQR--ARDAYinqlqtratdvdl 571
gi 74709301   32 CW-LRQA---KNGRCQ----VLYKTELS-Ke-----ECCSTGRLStSWt------------------------------- 66
gi 23821549   36 CWlQQGKE----ATCS----LVLKTQVS-Re-----ECCASGNINtAWs------------------------------- 70
gi 82087463   26 CW--YEQG--KNGRCQ----MLYLSEMS-Re-----DCCRSERLGmAWt------------------------------- 60
gi 123891431  32 CW-LQQSK---NGRCR------ELLKMN-Vsq---qECCRSGRLGsay-------------------------------- 65
gi 205591064  35 CW-LQQGK---NGRCQ----VLYMPGMS-Re-----ECCRSGRLGtSWt------------------------------- 69
gi 122102926 214 CW-QTHLGs---GKCG----QVFSTDIS-Rs----eCCGSSQSFSyt--------------------------------- 247
                         90       100       110
                 ....*....|....*....|....*....|....*...
3AIH_A        56 ---------------asKQ-HRLKrYHSQTYGNGSK-C 76
gi 74644875  610 nlaaiqdddgnmqhyvfGNlNELNnGLVLEYENGDQ-C 646
gi 74605164  572 nhlssdtveigkeeillGNlPDLNnGLILEYRGGTK-C 608
gi 74691767  572 ighlsnyspgaghdlllGNlPDIHnGLLLSYGNGHK-C 608
gi 74709301   67 ----------------eEDvNDNTlFKWMIFNGGAPnC 88
gi 23821549   71 -----------------NFtHPGNkISLLGFLGLVH-C 90
gi 82087463   61 ----------------eEDvPSNTlFRWLLMHGGAPnC 82
gi 123891431  66 ---------------tgEQvSTATlFRWMAFSGGAPnC 88
gi 205591064  70 ----------------eEDvPNSTlFRWMIFNGGAPnC 91
gi 122102926 248 ----------------dRElSSVEyFFATAIGGGVE-C 268
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