NP_766599.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
50,906 Da
NCBI Official Full Name
isocitrate dehydrogenase
NCBI Official Synonym Full Names
isocitrate dehydrogenase 2 (NADP+), mitochondrial
NCBI Protein Information
isocitrate dehydrogenase [NADP], mitochondrial
UniProt Protein Name
Isocitrate dehydrogenase [NADP], mitochondrial
UniProt Synonym Protein Names
ICD-M; IDP; NADP(+)-specific ICDH; Oxalosuccinate decarboxylase
UniProt Synonym Gene Names
UniProt Entry Name
IDHP_MOUSE
UniProt Comments for IDH2
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.
Protein type: Oxidoreductase; EC 1.1.1.42; Carbohydrate Metabolism - citrate (TCA) cycle; Mitochondrial; Other Amino Acids Metabolism - glutathione
Cellular Component: mitochondrion; mitochondrial inner membrane
Molecular Function: metal ion binding; magnesium ion binding; isocitrate dehydrogenase (NADP+) activity; oxidoreductase activity; NAD binding; oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Biological Process: isocitrate metabolic process; glyoxylate cycle; tricarboxylic acid cycle
Research Articles on IDH2
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Products associated with IDH2 sirna
Pathways associated with IDH2 sirna
Diseases associated with IDH2 sirna
Organs/Tissues associated with IDH2 sirna
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