Reactome | SGSH hydrolyses Heparan sulfate chain(2)

SGSH hydrolyses Heparan sulfate chain(2)

Stable Identifier

R-HSA-1678708

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

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SGSH hydrolyses Heparan sulfate chain(2)

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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
9285796	Novel mutations in Sanfilippo A syndrome: implications for enzyme function Weber, B, Guo, XH, Wraith, JE, Cooper, A, Kleijer, WJ, Bunge, S, Hopwood, JJ	Hum Mol Genet	1997
7493035	Cloning of the sulphamidase gene and identification of mutations in Sanfilippo A syndrome Scott, HS, Blanch, L, Guo, XH, Freeman, C, Orsborn, A, Baker, E, Sutherland, GR, Morris, CP, Hopwood, JJ	Nat Genet	1995