NAGLU hydrolyses Heparan sulfate chain(4)
GlcNAc-IdoA2S-GlcNS3S- group + H2O → GlcNAc + IdoA2S-GlcNS3S- group
- Reactome
- R-HSA-1678742
- Compartment
- lysosomal lumen
- Rhea
- None recorded
Inputs
- GlcNAc-IdoA2S-GlcNS3S- groupCHEBI:63807, C43H69N2O44S3, lysosomal lumen
- H2OCHEBI:15377, H2O, lysosomal lumen
Outputs
- GlcNAcCHEBI:17411, C8H15NO6, lysosomal lumen
- IdoA2S-GlcNS3S- groupCHEBI:63808, C35H56NO39S3, lysosomal lumen
Catalysed by
- alpha-N-acetylglucosaminidase activity of NAGLU(59-743) [lysosomal lumen]EC 3.2.1.50; UniProt P54802
Literature
- Cloning and expression of the gene involved in Sanfilippo B syndrome (mucopolysaccharidosis III B) Hum Mol Genet, 1996
- Molecular defects in Sanfilippo syndrome type B (mucopolysaccharidosis IIIB) J Inherit Metab Dis, 2005