Reactome | NAGLU hydrolyses Heparan sulfate chain(4)

NAGLU hydrolyses Heparan sulfate chain(4)

Stable Identifier

R-HSA-1678742

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

ReviewStatus

5/5

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NAGLU hydrolyses Heparan sulfate chain(4)

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Alpha-N-acetylglucosaminidase (NAGLU) hydrolyses the non-reducing, terminal N-acetyl-D-glucosamine residue from heparan sulfate. The active form of the enzyme (77kDa) is derived from a 82kDa precursor (Weber et al. 1996). Defects in NAGLU cause of mucopolysaccharidosis type IIIB (MPSIIIB, MIM:252920) also known as Sanfilippo syndrome type B (Beesley et al. 2005).

Literature References

PubMed ID	Title	Journal	Year
8776591	Cloning and expression of the gene involved in Sanfilippo B syndrome (mucopolysaccharidosis III B) Blanch, L, Weber, B, Hopwood, JJ, Scott, HS, Clements, PR	Hum Mol Genet	1996
16151907	Molecular defects in Sanfilippo syndrome type B (mucopolysaccharidosis IIIB) Beesley, CE, Young, EP, Winchester, BG, Vellodi, A, Jackson, M	J Inherit Metab Dis	2005