NP_001276839.1
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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
45,180 Da
NCBI Official Full Name
isocitrate dehydrogenase
NCBI Official Synonym Full Names
isocitrate dehydrogenase (NADP(+)) 2, mitochondrial
NCBI Official Synonym Symbols
IDH; IDP; IDHM; IDPM; ICD-M; D2HGA2; mNADP-IDH [Similar Products]
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
NCBI Summary for IDH2
Isocitrate dehydrogenases catalyze the oxidative decarboxylation of isocitrate to 2-oxoglutarate. These enzymes belong to two distinct subclasses, one of which utilizes NAD(+) as the electron acceptor and the other NADP(+). Five isocitrate dehydrogenases have been reported: three NAD(+)-dependent isocitrate dehydrogenases, which localize to the mitochondrial matrix, and two NADP(+)-dependent isocitrate dehydrogenases, one of which is mitochondrial and the other predominantly cytosolic. Each NADP(+)-dependent isozyme is a homodimer. The protein encoded by this gene is the NADP(+)-dependent isocitrate dehydrogenase found in the mitochondria. It plays a role in intermediary metabolism and energy production. This protein may tightly associate or interact with the pyruvate dehydrogenase complex. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Feb 2014]
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: Carbohydrate Metabolism - citrate (TCA) cycle; EC 1.1.1.42; Mitochondrial; Other Amino Acids Metabolism - glutathione; Oxidoreductase
Chromosomal Location of Human Ortholog: 15q26.1
Cellular Component: cytosol; extracellular exosome; mitochondrial inner membrane; mitochondrial matrix; mitochondrion; peroxisome
Molecular Function: isocitrate dehydrogenase (NADP+) activity; magnesium ion binding; NAD binding
Biological Process: 2-oxoglutarate metabolic process; carbohydrate metabolic process; glyoxylate cycle; isocitrate metabolic process; mitochondrion organization; NADP biosynthetic process; negative regulation of glial cell migration; negative regulation of matrix metallopeptidase secretion; tricarboxylic acid cycle
Disease: D-2-hydroxyglutaric Aciduria 2
Research Articles on IDH2
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Products associated with anti-IDH2 antibody
Pathways associated with anti-IDH2 antibody
Diseases associated with anti-IDH2 antibody
Disease Name |
Pubmed Publications |
Leukemia, Myeloid, Acute Antibodies |
>183 publications with IDH2 and Leukemia, Myeloid, Acute |
Nervous System Diseases Antibodies |
>156 publications with IDH2 and Nervous System Diseases |
Brain Diseases Antibodies |
>150 publications with IDH2 and Brain Diseases |
Oligodendroglioma Antibodies |
>38 publications with IDH2 and Oligodendroglioma |
Cell Transformation, Neoplastic Antibodies |
>29 publications with IDH2 and Cell Transformation, Neoplastic |
Cholangiocarcinoma Antibodies |
>19 publications with IDH2 and Cholangiocarcinoma |
Neoplasms, Experimental Antibodies |
>12 publications with IDH2 and Neoplasms, Experimental |
Liver Neoplasms Antibodies |
>12 publications with IDH2 and Liver Neoplasms |
Anemia Antibodies |
>10 publications with IDH2 and Anemia |
Lymphoma, T-Cell, Peripheral Antibodies |
>8 publications with IDH2 and Lymphoma, T-Cell, Peripheral |
Organs/Tissues associated with anti-IDH2 antibody
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