LMPD Database

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LMP002748

UniProt Annotations

Entry Information
Gene NameN-acetylgalactosaminidase, alpha-
Protein EntryNAGAB_HUMAN
UniProt IDP17050
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityCleavage of non-reducing alpha-(1->3)-N- acetylgalactosamine residues from human blood group A and AB mucin glycoproteins, Forssman hapten and blood group A lacto series glycolipids.
DiseaseKanzaki disease (KANZD) [MIM
DiseaseSchindler disease (SCHIND) [MIM
FunctionRemoves terminal alpha-N-acetylgalactosamine residues from glycolipids and glycopeptides. Required for the breakdown of glycolipids.
MiscellaneousAlpha-galactosidase B was first found to be an isoenzyme of alpha-galactosidases, but apparently it differs from alpha-galactosidase A in substrate specificity and is alpha-N- acetylgalactosaminidase.
Sequence CautionSequence=AAA59902.1; Type=Frameshift; Positions=320; Evidence= ;
SimilarityBelongs to the glycosyl hydrolase 27 family.
Subcellular LocationLysosome.
SubunitHomodimer.