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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.

ECHS1 hydrates methacrylyl-CoA

MACR-CoA + H2O → bHIB-CoA

Reactome: R-HSA-70870
Compartment: mitochondrial matrix
Rhea: RHEA:31176

Inputs

MACR-CoACHEBI:62500, C25H36N7O17P3S, mitochondrial matrix
H2OCHEBI:15377, H2O, mitochondrial matrix

Outputs

bHIB-CoACHEBI:62611, C25H38N7O18P3S, mitochondrial matrix

Catalysed by

enoyl-CoA hydratase activity of ECHS1 hexamer [mitochondrial matrix]EC 4.2.1.17

Literature

Clinical, biochemical and metabolic characterisation of a mild form of human short-chain enoyl-CoA hydratase deficiency: significance of increased N-acetyl-S-(2-carboxypropyl)cysteine excretion J. Med. Genet., 2015