Alpha-L-iduronidase (IDUA) hydrolyses the unsulfated alpha-L-iduronosidic linkage in DS Authored: Jassal, B, 2011-10-21 EC Number: 3.2.1.76 Edited: Jassal, B, 2011-10-21 Pubmed1946389 Pubmed8213840 Reactome Database ID Release 431793186 Reactome, http://www.reactome.org ReactomeREACT_121062 Reviewed: D'Eustachio, P, 2012-03-28 The lysosomal enzyme alpha-L-iduronidase (IDUA) hydrolyzes the nonreducing terminal iduronide glycosidic bond in heparan sulfate and dermatan sulfate (Scott et al. 1991). Defects in IDUA cause mucopolysaccharidosis type IH (MIM:607014, also called Hurler syndrome), mucopolysaccharidosis type IH/S (MIM:607015, also called HurlerScheie syndrome) and mucopolysaccharidosis type IS (MIM:607016, also called Scheie syndrome) (Scott et al. 1993).