Reactome | NAGLU hydrolyses heparan chain(2)

NAGLU hydrolyses heparan chain(2)

Stable Identifier

R-HSA-2090038

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

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Alpha-N-acetylglucosaminidase (NAGLU) also hydrolyses another non-reducing, 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
16151907	Molecular defects in Sanfilippo syndrome type B (mucopolysaccharidosis IIIB) Beesley, CE, Jackson, M, Young, EP, Vellodi, A, Winchester, BG	J Inherit Metab Dis	2005
8776591	Cloning and expression of the gene involved in Sanfilippo B syndrome (mucopolysaccharidosis III B) Weber, B, Blanch, L, Clements, PR, Scott, HS, Hopwood, JJ	Hum Mol Genet	1996