IDUA hydrolyses Heparan sulfate chain(6)

Stable Identifier
R-HSA-2090037
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
Alpha-L-iduronidase (IDUA) hydrolyses another unsulfated alpha-L-iduronosidic linkage in heparan sulfate
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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
Participants
Participates
Catalyst Activity

L-iduronidase activity of IDUA [lysosomal lumen]

Orthologous Events
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