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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.

beta-methylglutaconyl-CoA + H2O beta-hydroxy-beta-methylglutaryl-CoA

bMG-CoA + H2O → bHMG-CoA

Reactome: R-HSA-70785
Compartment: mitochondrial matrix
Rhea: RHEA:21538

Inputs

bMG-CoACHEBI:57346, C27H37N7O19P3S, mitochondrial matrix
H2OCHEBI:15377, H2O, mitochondrial matrix

Outputs

bHMG-CoACHEBI:43074, C27H39N7O20P3S, mitochondrial matrix

Catalysed by

methylglutaconyl-CoA hydratase activity of AUH hexamer [mitochondrial matrix]EC 4.2.1.18

Literature

3-Methylglutaconic aciduria type I is caused by mutations in AUH. Am J Hum Genet, 2002
Crystal structure of human AUH protein, a single-stranded RNA binding homolog of enoyl-CoA hydratase Structure, 2001
Deficiency of 3-methylglutaconyl-coenzyme A hydratase in two siblings with 3-methylglutaconic aciduria. J Clin Invest, 1986