SGSH hydrolyses Heparan sulfate chain(2)

Stable Identifier
R-HSA-1678708
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
N-sulfoglucosamine sulfohydrolase activity of SGSH [lysosomal lumen]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created