Alpha-L-iduronidase (IDUA) hydrolyses the unsulfated alpha-L-iduronosidic linkage in heparan sulfate An L-iduronic acid residue can be cleaved from either heparan sulfate or dermatan sulfate by the lysosomal enzyme alpha-L-iduronidase (IDUA) (Scott et al. 1991). Defects in IDUA are the cause of mucopolysaccharidosis type IH (MPS IH, Hurler syndrome, MIM:607014), mucopolysaccharidosis IH/S (MPSIH/S, HurlerScheie syndrome, MIM:607015) and mucopolysaccharidosis type IS (MPSIS, Scheie syndrome, MIM:607016) (LeeChen et al. 1999). Authored: Jassal, B, 2011-10-19 EC Number: 3.2.1.76 Edited: Jassal, B, 2011-10-19 Pubmed10466419 Pubmed1946389 Reactome Database ID Release 431678716 Reactome, http://www.reactome.org ReactomeREACT_120763 Reviewed: D'Eustachio, P, 2012-03-28