MGP Database

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MGP004492

UniProt Annotations

Entry Information
Gene Namemannosidase, alpha, class 1B, member 1
Protein EntryMA1B1_HUMAN
UniProt IDQ9UKM7
SpeciesHuman
Comments
Comment typeDescription
Biophysicochemical PropertiesKinetic parameters: KM=0.4 mM for Man9GlcNAc2 {ECO:0000269|PubMed:10521544}; pH dependence: Optimum pH is between 6.5 and 6.9. {ECO:0000269|PubMed:10521544};
Catalytic ActivityHydrolysis of the terminal (1->2)-linked alpha-D-mannose residues in the oligo-mannose oligosaccharide Man(9)(GlcNAc)(2). {ECO:0000269|PubMed:10409699, ECO:0000269|PubMed:12090241, ECO:0000269|PubMed:15713668}.
CautionIt is uncertain whether Met-1 or Met-37 is the initiator. {ECO:0000305}.
CofactorName=Ca(2+); Xref=ChEBI:CHEBI:29108;
DiseaseMental retardation, autosomal recessive 15 (MRT15) [MIM:614202]: A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptive behavior and manifested during the developmental period. {ECO:0000269|PubMed:21763484}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Enzyme RegulationInhibited by both 1-deoxymannojirimycin (dMNJ) and kifunensine. {ECO:0000269|PubMed:10409699}.
FunctionInvolved in glycoprotein quality control targeting of misfolded glycoproteins for degradation. It primarily trims a single alpha-1,2-linked mannose residue from Man(9)GlcNAc(2) to produce Man(8)GlcNAc(2), but at high enzyme concentrations, as found in the ER quality control compartment (ERQC), it further trims the carbohydrates to Man(5-6)GlcNAc(2). {ECO:0000269|PubMed:12090241, ECO:0000269|PubMed:18003979}.
PathwayProtein modification; protein glycosylation.
SimilarityBelongs to the glycosyl hydrolase 47 family. {ECO:0000305}.
Subcellular LocationEndoplasmic reticulum membrane {ECO:0000269|PubMed:10409699}; Single-pass type II membrane protein {ECO:0000269|PubMed:10409699}.
Tissue SpecificityWidely expressed. {ECO:0000269|PubMed:10409699, ECO:0000269|PubMed:10521544}.
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