Beta-mannosidase

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