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Protein Page:
IDH2 (human)

IDH2 a mitochondrial oxidoreductase that catalyzes the third step of the citric acid cycle: the oxidative decarboxylation of isocitrate, consuming NADP(+), and producing alpha-ketoglutarate (alpha-KG) and CO2. Can also use oxalosuccinate as a substrate. Alpha-KG is an activator the dioxygenases that hydroxylate the transcription factor HIF and lead to its degradation by VHL. Since HIF turns on oncogenic pathways, IDH2 has apparent tumor suppressor activity. Homo-dimerization is required for activity. Each subunit binds 1 magnesium or manganese ion. May tightly associate or interact with the pyruvate dehydrogenase complex. A somatic mutation resulting in the R172K conversion results in a neomorphic enzyme activity that converts isocitrate to R(-)-2-hydroxyglutarate (2HG) occurs in a significant percentage of patients with cytogenetically normal acute myeloid leukemia (AML). Elevated levels of 2HG are also correlated with an elevated risk of malignant brain tumors. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; EC; Mitochondrial; Other Amino Acids Metabolism - glutathione; Oxidoreductase
Chromosomal Location of Human Ortholog: 15q26.1
Cellular Component: mitochondrial matrix; mitochondrion
Molecular Function: isocitrate dehydrogenase (NADP+) activity; magnesium ion binding
Biological Process: 2-oxoglutarate metabolic process; isocitrate metabolic process; tricarboxylic acid cycle
Disease: D-2-hydroxyglutaric Aciduria 2
Reference #:  P48735 (UniProtKB)
Alt. Names/Synonyms: ICD-M; IDH; IDH2; IDHM; IDHP; IDP; IDPM; isocitrate dehydrogenase 2 (NADP+), mitochondrial; Isocitrate dehydrogenase [NADP], mitochondrial; isocitrate dehydrogenase, mitochondrial; mNADP-IDH; NADP(+)-specific ICDH; NADP+-specific ICDH; Oxalosuccinate decarboxylase
Gene Symbols: IDH2
Molecular weight: 50,909 Da
Basal Isoelectric point: 8.88  Predict pI for various phosphorylation states
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