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Β-Mannosidase

β-Mannosidase (}, mannanase, mannase, β-<small>D</small>-mannosidase, β-mannoside mannohydrolase, exo-β-<small>D</small>-mannanase, lysosomal β A mannosidase) is an enzyme with systematic name β-<small>D</small>-mannoside mannohydrolase, which is in humans encoded by the MANBA gene. This enzyme catalyses the following chemical reaction

Hydrolysis of terminal, non-reducing β-<small>D</small>-mannose residues in β-<small>D</small>-mannosides

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 β-mannosidosis, a lysosomal storage disease that has a wide spectrum of neurological involvement.

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