Β-Mannosidase

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


 * Hydrolysis of terminal, non-reducing β- D -mannose residues in β- D -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.