Atlas One · Host Biology · Scale 03 — Molecular

IDH2

IDH2 (mitochondrial isocitrate dehydrogenase 2) catalyzes isocitrate → α-KG in the TCA cycle; IDH2 R140Q/R172K gain-of-function mutations produce 2-hydroxyglutarate → TET2/KDM inhibition → DNA hypermethylation; enasidenib is the approved IDH2 inhibitor for IDH2-mutant AML.

Also known as: IDH2, isocitrate dehydrogenase 2, IDH2 R140Q, IDH2 R172K, 2-hydroxyglutarate, 2-HG, enasidenib, IDH2-mutant AML, oncometabolite

Scale 03 — MolecularScale
draftStatus
2026-06-06Last Reviewed
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idh2

IDH2

IDH2 (mitochondrial isocitrate dehydrogenase 2) catalyzes isocitrate → α-KG in the TCA cycle; IDH2 R140Q/R172K gain-of-function mutations produce 2-hydroxyglutarate → TET2/KDM inhibition → DNA hypermethylation; enasidenib is the approved IDH2 inhibitor for IDH2-mutant AML.

Entry Metadata

FieldValue
IDidh2
NameIDH2
Statusdraft
Last reviewed2026-06-06
Atlas01-human
Scale03-molecular

Cross-Atlas Connections

connects-toIdh1 connects-toFgfr connects-toMtor connects-toP53

Sources