IDUA hydrolyses Heparan sulfate chain(6)

Stable Identifier
R-HSA-2090037
Type
Reaction [transition]
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
1946389 Human alpha-L-iduronidase: cDNA isolation and expression

Scott, HS, Anson, DS, Orsborn, AM, Nelson, PV, Clements, PR, Morris, CP, Hopwood, JJ

Proc Natl Acad Sci U S A 1991
10466419 Mucopolysaccharidosis type I: characterization of novel mutations affecting alpha-L-iduronidase activity

Lee-Chen, GJ, Lin, SP, Tang, YF, Chin, YW

Clin Genet 1999
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
L-iduronidase activity of IDUA [lysosomal lumen]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created