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Human metabolism

Reactome's human metabolism drawn as one network. Each molecule has one home node; widely shared molecules also appear as dashed copies in other territories, linked from their details. .

Territories

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Metabolite; its structure appears as you zoom in
Reaction, arrow toward the product
Cofactors and co-substrates are written beside the reaction when you zoom in
Dashed ring: a copy of a widely shared molecule or cofactor in another territory or pathway
Compartment transfer of the molecule it is linked to
Binding, release or complex event; its proteins are named in its details
Outline: reactions mostly in the mitochondrion

Arrows follow Reactome's curated reaction direction.

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

H2O + IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl → aldehydo-L-iduronic acid + CHEBI:63868 chain

Reactome: R-HSA-1793186
Compartment: lysosomal lumen
Rhea: None recorded

Inputs

H2OCHEBI:15377, H2O, lysosomal lumen
IdoA-GalNAc(4S)-GlcA-Gal-Gal-XylCHEBI:63873, C37H59NO35S, lysosomal lumen

Outputs

aldehydo-L-iduronic acidCHEBI:21338, C6H9O7, lysosomal lumen
CHEBI:63868 chainCHEBI:63868, C31H51NO29S, lysosomal lumen

Catalysed by

L-iduronidase activity of IDUA [lysosomal lumen]EC 3.2.1.76; UniProt P35475

Literature

Human alpha-L-iduronidase: cDNA isolation and expression Proc Natl Acad Sci U S A, 1991
Identification of mutations in the alpha-L-iduronidase gene (IDUA) that cause Hurler and Scheie syndromes Am J Hum Genet, 1993