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alpha D Mannoside Mannohydrolase

An enzyme that catalyzes the Hydrolysis of terminal, non-reducing alpha-D-Mannose residues in alpha-D-Mannosides. The enzyme plays a Role in the processing of newly formed N-glycans and in degradation of mature Glycoproteins. There are multiple Isoforms of alpha-mannosidase, each having its own specific cellular location and pH optimum. Defects in the lysosomal form of the enzyme results in a buildup of mannoside intermediate metabolites and the Disease alpha-Mannosidosis.