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N-sulphoglucosamine sulphohydrolase is a lysosomal enzyme that catalyzes the removal of sulfate groups from glucosamine residues in heparan sulfate. It is essential for the degradation of glycosaminoglycans (GAGs) within lysosomes. Mutations in SGSH lead to mucopolysaccharidosis type IIIA (MPS IIIA), also known as Sanfilippo syndrome type A, a severe lysosomal storage disorder.
Hydrolyzes terminal N-sulfated glucosamines on heparan sulfate, removing sulfate groups.
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