Reactome | SGSH hydrolyses Heparan sulfate chain(7)

SGSH hydrolyses Heparan sulfate chain(7)

Stable Identifier

R-HSA-2090043

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

ReviewStatus

5/5

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SGSH hydrolyses Heparan sulfate chain(7)

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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