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: 400321
View PSSM: pfam07915
Aligned: 100 rows
Threshold Bit Score: 30.9741
Threshold Setting Gi: 74610511
Created: 21-May-2020
Updated: 7-Aug-2020
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:
3AIH_A   3 APCL-LKTKDWWTYEFCY----GRHIQQYHMe--DSEIKGEVLYLG-YY-------QSaFDWDDETAKAS---------- 57  human
Q04924 537 SYCL-DDILDNYVYTICF----QRPMTEGVIyqaEDKVDGKKVLIG-RFktsgfnvDLnMEKYAEHLKATydeksdlisn 610 Saccharomyces cerevis...
Q6CK66 502 DFCV-DSELNSYIYRLCYsndkGGDIVQIEN-----KPDGNKVVIG-RYrgf--tiDTnSKYDVYLQSLKyrypntevgn 572 Kluyveromyces lactis
Q74ZM0 505 DFCI-SSKFDEYTYHICF----NPDTGRILQ---VQDRDRSVVLVG-HFkd---fkLVsQRARDAYINQLqtratdvdli 572 Eremothecium gossypii
Q6FHE1  30 GNCW-LRQA---KNGRCQ----VLYKTELSK-----EECCSTGRLStSWte----------------------------- 67  human
Q99PW7  34 GVCWlQQGKE----ATCS----LVLKTQVSR-----EECCASGNINtAWs------------------------------ 70  Norway rat
Q6PW72  24 GNCW--YEQG--KNGRCQ----MLYLSEMSR-----EDCCRSERLGmAWte----------------------------- 61  blue catfish
Q20CF4  30 GNCW-LQQSK---NGRCR------ELLKMNV---SQQECCRSGRLGsay------------------------------- 65  sea lamprey
Q9YHV4  33 GNCW-LQQGK---NGRCQ----VLYMPGMSR-----EECCRSGRLGtSWte----------------------------- 70  zebrafish
Q86NV3 212 GTCW-QTHLGs---GKCG----QVFSTDISR----SECCGSSQSFSyt-------------------------------- 247 fruit fly
3AIH_A  58 -----------------KQHRLKRYHSQTYGNGSK-C 76  human
Q04924 611 laaiqdddgnmqhyvfgNLNELNNGLVLEYENGDQ-C 646 Saccharomyces cerevisiae S288C
Q6CK66 573 hlssdtveigkeeillgNLPDLNNGLILEYRGGTK-C 608 Kluyveromyces lactis
Q74ZM0 573 ghlsnyspgaghdlllgNLPDIHNGLLLSYGNGHK-C 608 Eremothecium gossypii
Q6FHE1  68 ----------------eDVNDNTLFKWMIFNGGAPnC 88  human
Q99PW7  71 ----------------nFTHPGNKISLLGFLGLVH-C 90  Norway rat
Q6PW72  62 ----------------eDVPSNTLFRWLLMHGGAPnC 82  blue catfish
Q20CF4  66 --------------tgeQVSTATLFRWMAFSGGAPnC 88  sea lamprey
Q9YHV4  71 ----------------eDVPNSTLFRWMIFNGGAPnC 91  zebrafish
Q86NV3 248 ---------------drELSSVEYFFATAIGGGVE-C 268 fruit fly
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