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IDH1
an oxidoreductase that catalyzes the third step of the TCA cycle: the oxidative decarboxylation of isocitrate, consuming NADP(+), and producing alpha-ketoglutarate (alpha-KG) and CO2. 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, IDH1 has apparent tumor suppressor activity. Homo-dimerization is required for activity. Each subunit binds 1 magnesium or manganese ion. IDH1 is located in the cytosol and peroxisomes. Somatic mutations affecting arginine 132 (R132) are found in 80% of grade II?III gliomas and secondary glioblastomas in humans, and cause disease in a tissue-specific fashion. Only a single copy of the gene has been found to be mutated in tumours. Mutations of R132 to H, C, S, G, V, or L have been reported to be neomorphic, abolishing the conversion of isocitrate to alpha-KG. Instead, alpha-KG is converted to R(-)-2-hydroxyglutarate (2HG). Elevated levels of 2HG correlate with an elevated risk of malignant brain tumors. Neomorphic mutations in IDH1 and IDH2 convert alpha-KG to 2-hydroxyglutarate (2-HG) occur in a high percentage of patients with cytogenetically normal acute myeloid leukemia (AML). Note: This description may include information from UniProtKB.
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| Protein type: EC 1.1.1.42; Oxidoreductase; Other Amino Acids Metabolism - glutathione; Carbohydrate Metabolism - citrate (TCA) cycle |
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Cellular Component: peroxisomal matrix; mitochondrion; cytoplasm; peroxisome; cytosol
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Molecular Function: protein homodimerization activity; magnesium ion binding; isocitrate dehydrogenase (NADP+) activity; NAD binding; receptor binding
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Biological Process: glyoxylate cycle; NADPH regeneration; isocitrate metabolic process; glutathione metabolic process; tricarboxylic acid cycle; cellular lipid metabolic process; response to oxidative stress; 2-oxoglutarate metabolic process
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Reference #:
O75874 (UniProtKB)
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| Alt. Names/Synonyms: Cytosolic NADP-isocitrate dehydrogenase; IDCD; IDH; IDH1; IDHC; IDP; IDPC; isocitrate dehydrogenase 1 (NADP+), soluble; Isocitrate dehydrogenase [NADP] cytoplasmic; NADP(+)-specific ICDH; NADP+-specific ICDH; NADP-dependent isocitrate dehydrogenase, cytosolic; NADP-dependent isocitrate dehydrogenase, peroxisomal; Oxalosuccinate decarboxylase; PICD |
| Gene Symbols: IDH1 |
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Molecular weight: 46,659 Da
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Basal Isoelectric point: 6.53
Predict pI for various phosphorylation states
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Protein-Specific Antibodies or siRNAs from Cell Signaling Technology®
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