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