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

Stable Identifier
R-HSA-1793186
Type
Reaction
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
Participants
Participates
Catalyst Activity

L-iduronidase activity of IDUA [lysosomal lumen]

Orthologous Events
Authored
Reviewed
Created
Cite Us!