MANB1; Mannosidase Beta

MANBA is a lysosomal beta-mannosidase enzyme that catalyzes the hydrolysis of beta-1,4-linked mannose residues from oligosaccharides. It is crucial for the degradation of N-linked glycans within lysosomes. Deficiency in MANBA leads to beta-mannosidosis, a rare lysosomal storage disease characterized by the accumulation of mannose-containing oligosaccharides, resulting in neurological and other systemic abnormalities.