N-sulphoglucosamine sulphohydrolase (SGSH) hydrolyses another sulfate from the N-sulphoglucosamine residue of heparan sulfate Alpha-N-acetylglucosaminidase (NAGLU) also hydrolyses another nonreducing, 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). Authored: Jassal, B, 2012-02-06 EC Number: 3.10.1.1 Edited: Jassal, B, 2012-02-06 Pubmed7493035 Pubmed9285796 Reactome Database ID Release 432090043 Reactome, http://www.reactome.org ReactomeREACT_121158 Reviewed: D'Eustachio, P, 2012-03-28 has a Stoichiometric coefficient of 2