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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.
Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; EC; Other Amino Acids Metabolism - glutathione; Oxidoreductase
Chromosomal Location of Human Ortholog: 2q34
Cellular Component: cytoplasm; cytosol; extracellular exosome; extracellular region; ficolin-1-rich granule lumen; mitochondrion; peroxisomal matrix; peroxisome; secretory granule lumen; tertiary granule lumen
Molecular Function: (R)-2-hydroxyglutarate dehydrogenase activity; cadherin binding; identical protein binding; isocitrate dehydrogenase (NADP+) activity; magnesium ion binding; NAD binding; NADP binding; protein homodimerization activity; receptor binding
Biological Process: 2-oxoglutarate metabolic process; female gonad development; glutathione metabolic process; glyoxylate cycle; isocitrate metabolic process; NADPH regeneration; neutrophil degranulation; response to oxidative stress; response to steroid hormone; tricarboxylic acid cycle
Disease: Glioma Susceptibility 1
Reference #:  O75874 (UniProtKB)
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
Molecular weight: 46,659 Da
Basal Isoelectric point: 6.53  Predict pI for various phosphorylation states
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