SGSH hydrolyses Heparan sulfate chain(7)

Stable Identifier
R-HSA-2090043
Type
Reaction
Species
Homo sapiens
Compartment
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Alpha-N-acetylglucosaminidase (NAGLU) also hydrolyses another nonreducing, terminal N-acetyl-D-glucosamine residue from heparan sulfate. The active form of the enzyme (77kDa) is derived from an 82kDa precursor (Weber et al. 1996). Defects in NAGLU cause Mucopolysaccharidosis type IIIB (MPSIIIB, MIM:252920), also known as Sanfilippo syndrome type B (Beesley et al. 2005).

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]

Orthologous Events
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