Reactome | IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS

IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS

Stable Identifier

R-HSA-1793186

Type

Reaction

Species

Homo sapiens

Compartment

lysosomal lumen

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IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS

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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).

Literature References

PubMed ID	Title	Journal	Year
1946389	Human alpha-L-iduronidase: cDNA isolation and expression Morris, CP, Orsborn, AM, Nelson, PV, Hopwood, JJ, Anson, DS, Scott, HS, Clements, PR	Proc Natl Acad Sci U S A	1991
8213840	Identification of mutations in the alpha-L-iduronidase gene (IDUA) that cause Hurler and Scheie syndromes Morris, CP, Thompson, PR, Nelson, PV, Hopwood, JJ, Scott, HS, Brooks, DA, Litjens, T	Am J Hum Genet	1993