N-sulphoglucosamine sulphohydrolase (SGSH) hydrolyses the sulfate group from the terminal N-sulphoglucosamine residue of heparan sulfate (Scott et al. 1995). Defects in SGSH cause mucopolysaccharidosis type IIIA (MPSIIIA, MIM:252900), also called Sanfilippo syndrome A (Weber et al. 1997).
Weber, B, Guo, XH, Wraith, JE, Cooper, A, Kleijer, WJ, Bunge, S, Hopwood, JJ
Scott, HS, Blanch, L, Guo, XH, Freeman, C, Orsborn, A, Baker, E, Sutherland, GR, Morris, CP, Hopwood, JJ
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