NAGLU hydrolyses Heparan sulfate chain(4)

Stable Identifier
R-HSA-1678742
Type
Reaction
Species
Homo sapiens
Compartment
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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
Participants
Participates
Catalyst Activity

alpha-N-acetylglucosaminidase activity of NAGLU(59-743) [lysosomal lumen]

Orthologous Events
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