SGSH hydrolyses Heparan sulfate chain(2)

Stable Identifier
R-HSA-1678708
Type
Reaction
Species
Homo sapiens
Compartment
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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).

Literature References
PubMed ID Title Journal Year
7493035 Cloning of the sulphamidase gene and identification of mutations in Sanfilippo A syndrome

Blanch, L, Morris, CP, Orsborn, A, Guo, XH, Hopwood, JJ, Sutherland, GR, Freeman, C, Scott, HS, Baker, E

Nat Genet 1995
9285796 Novel mutations in Sanfilippo A syndrome: implications for enzyme function

Weber, B, Bunge, S, Guo, XH, Hopwood, JJ, Cooper, A, Kleijer, WJ, Wraith, JE

Hum Mol Genet 1997
Participants
Participates
Catalyst Activity

N-sulfoglucosamine sulfohydrolase activity of SGSH [lysosomal lumen]

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