Reactome | IDUA hydrolyses Heparan sulfate chain(1)

IDUA hydrolyses Heparan sulfate chain(1)

Stable Identifier

R-HSA-1678716

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

ReviewStatus

5/5

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IDUA hydrolyses Heparan sulfate chain(1)

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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).

Literature References

PubMed ID	Title	Journal	Year
10466419	Mucopolysaccharidosis type I: characterization of novel mutations affecting alpha-L-iduronidase activity Lee-Chen, GJ, Chin, YW, Tang, YF, Lin, SP	Clin Genet	1999
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