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).
Morris, CP, Orsborn, AM, Nelson, PV, Hopwood, JJ, Anson, DS, Scott, HS, Clements, PR
Morris, CP, Thompson, PR, Nelson, PV, Hopwood, JJ, Scott, HS, Brooks, DA, Litjens, T
L-iduronidase activity of IDUA [lysosomal lumen]
© 2022 Reactome