Reaction: NAGLU hydrolyses Heparan sulfate chain(4)
- in pathway: HS-GAG degradation
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).
Reaction - small molecule participants:
Heparan sulfate chain(5) [lysosomal lumen]
GlcNAc [lysosomal lumen]
H2O [lysosomal lumen]
Heparan sulfate chain(4) [lysosomal lumen]
Reactome.org reaction link: R-HSA-1678742
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Reaction input - small molecules:
water
alpha-D-GlcNAc-(1->4)-beta-D-IdoA2S-(1->4)-alpha-D-GlcNS3S-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl-yl group
Reaction output - small molecules:
beta-D-IdoA2S-(1->4)-alpha-D-GlcNS3S-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl-yl group
aldehydo-N-acetyl-D-glucosamine
Reactome.org link: R-HSA-1678742