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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.

BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV

Lipo-K105-DBT + KIC, KMVA, KIV + H+ → CO2 + BCAA-lipoH2-K105-DBT

Reactome: R-HSA-9859148
Compartment: mitochondrial matrix
Rhea: RHEA:13458

Inputs

Lipo-K105-DBTR-HSA-70017, mitochondrial matrix
KIC, KMVA, KIVR-ALL-508181, mitochondrial matrix
H+CHEBI:15378, H, mitochondrial matrix

Outputs

CO2CHEBI:16526, CO2, mitochondrial matrix
BCAA-lipoH2-K105-DBTR-HSA-9859149, mitochondrial matrix

Catalysed by

oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor of BCKDH [mitochondrial matrix]EC 1.2.4

Literature

Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease. Structure Fold Des, 2000