Skip to content

DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap

Data

Loading the map…

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

Select a territory to bring it forward.

Reading the map

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.

isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2

ISV-CoA + FAD + H+ → bMC-CoA + FADH2

Reactome: R-HSA-70745
Compartment: mitochondrial matrix
Rhea: RHEA:12277

Inputs

ISV-CoACHEBI:57345, C26H40N7O17P3S, mitochondrial matrix
FADCHEBI:57692, C27H30N9O15P2, mitochondrial matrix
H+CHEBI:15378, H, mitochondrial matrix

Outputs

bMC-CoACHEBI:57344, C26H38N7O17P3S, mitochondrial matrix
FADH2CHEBI:58307, C27H33N9O15P2, mitochondrial matrix

Catalysed by

3-methylbutanoyl-CoA dehydrogenase activity of IVD tetramer [mitochondrial matrix]EC 1.3.8.4

Literature

Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver. J Biol Chem, 1987
Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia. Proc Natl Acad Sci U S A, 1980
Structure of human isovaleryl-CoA dehydrogenase at 2.6 A resolution: structural basis for substrate specificity,. Biochemistry, 1997