| UniProt Protein Function: | 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). Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; EC 1.1.1.42; Other Amino Acids Metabolism - glutathione; OxidoreductaseChromosomal Location of Human Ortholog: 1 C2|1 32.91 cMCellular Component: cytoplasm; cytosol; mitochondrion; peroxisomeMolecular Function: (R)-2-hydroxyglutarate dehydrogenase activity; cadherin binding; isocitrate dehydrogenase (NADP+) activity; isocitrate dehydrogenase activity; magnesium ion binding; NADP binding; protein homodimerization activity; receptor bindingBiological Process: 2-oxoglutarate metabolic process; glutathione metabolic process; isocitrate metabolic process; response to organic cyclic substance; response to oxidative stress; response to steroid hormone stimulus |
| UniProt Protein Details: | |
| NCBI Summary: | |
| UniProt Code: | O88844 |
| NCBI GenInfo Identifier: | 162417977 |
| NCBI Gene ID: | 15926 |
| NCBI Accession: | NP_001104790.1 |
| UniProt Secondary Accession: | O88844,Q3UAV7 |
| UniProt Related Accession: | O88844 |
| Molecular Weight: | 49.2kD |
| NCBI Full Name: | isocitrate dehydrogenase |
| NCBI Synonym Full Names: | isocitrate dehydrogenase 1 (NADP+), soluble |
| NCBI Official Symbol: | Idh1Â Â |
| NCBI Official Synonym Symbols: | Id-1; Idpc; Idh-1; AI314845; AI788952; E030024J03Rik  |
| NCBI Protein Information: | isocitrate dehydrogenase [NADP] cytoplasmic |
| UniProt Protein Name: | Isocitrate dehydrogenase [NADP] cytoplasmic |
| UniProt Synonym Protein Names: | Cytosolic NADP-isocitrate dehydrogenase; IDP; NADP(+)-specific ICDH; Oxalosuccinate decarboxylase |
| Protein Family: | Isocitrate dehydrogenase |
| UniProt Gene Name: | Idh1Â Â |
| UniProt Entry Name: | |
