Isocitrate Dehydrogenase 1, Soluble (IDH1) Antibody – Rabbit Polyclonal

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; Oxidoreductase

Chromosomal Location of Human Ortholog: 9q32

Cellular Component: cytoplasm; cytosol; mitochondrion; peroxisome

Molecular Function: (R)-2-hydroxyglutarate dehydrogenase activity; cadherin binding; isocitrate dehydrogenase (NADP+) activity; isocitrate dehydrogenase 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; response to organic cyclic compound; response to oxidative stress; response to steroid hormone; tricarboxylic acid cycle