Reactome | IDUA cleaves iduronate from HS chain

IDUA cleaves iduronate from HS chain

Stable Identifier

R-HSA-1678716

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

Synonyms

IDUA hydrolyses Heparan sulfate chain(1)

ReviewStatus

5/5

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The lysosomal enzyme alpha-L-iduronidase (IDUA) cleaves the terminal L-iduronic acid residue from either heparan sulfate or dermatan sulfate chains of proteoglycans (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). The exact details of how free iduronic acid is further processed in lysosomes remain unclear and need further research.

Literature References

PubMed ID	Title	Journal	Year
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
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