Tags: Protein.
N-sulphoglucosamine sulphohydrolase is an enzyme that in humans is encoded by the SGSH gene.A number sign (#) is used with this entry because the phenotype is caused by mutation in the gene encoding N-sulfoglucosamine sulfohydrolase (SGSH; MIM 605270). The Sanfilippo syndrome or mucopolysaccharidosis III is a lysosomal storage disease due to impaired degradation of heparan sulfate (Esposito et al. 2000 [PubMed 10727844]).