This gene encodes a member of the glycosyl hydrolase 2 family. The encoded protein localizes to the lysosome where it is the final exoglycosidase in the pathway for N-linked glycoprotein oligosaccharide catabolism. Mutations in this gene are associated with beta-mannosidosis, a lysosomal storage disease that has a wide spectrum of neurological involvement.
Pathway
Lysosome, organism-specific biosystem; Lysosome, conserved biosystem; Other glycan degradation, organism-specific biosystem; Other glycan degradation, conserved biosystem;
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