IDH3 complex decarboxylates isocitrate

Stable Identifier
R-HSA-70967
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
D-threo-isocitrate + NAD(+) => 2-oxoglutarate + CO2 + NADH
ReviewStatus
5/5
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Mitochondrial isocitrate dehydrogenase IDH3 catalyzes the irreversible reaction of isocitrate and NAD+ to form alpha-ketoglutarate, CO2, and NADH. The enzyme is a heterooctamer containing two copies of a heterotetramer of two IDH3A, one IDH3B, one IDH3G, and two Mn++ (PDB 7CE3; Sun et al., 2020; Dange and Colman, 2010). It is activated by ADP (Soundar et al., 2003, 2006; Bzymek and Colman, 2007) and inhibited by NADH and high concentrations of ATP (Cohen & Colman, 1972; Sun et al., 2020). This is the first of four oxidation reactions in the citric acid cycle and the first decarboxylation.

Mutations in the IDH3A, IDH3B gene can cause retinitis pigmentosa (RP46; MIM:612572; RP90; MIM:619007) (Hartong et al., 2008).
Participants
Participates
Catalyst Activity

isocitrate dehydrogenase (NAD+) activity of IDH3 octamer [mitochondrial matrix]

This event is regulated
Negatively by
Orthologous Events
Cross References
Rhea
RHEA
Reviewed
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