NP_113698.1
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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
46,734 Da
NCBI Official Full Name
isocitrate dehydrogenase
NCBI Official Synonym Full Names
isocitrate dehydrogenase 1 (NADP+), soluble
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
UniProt Synonym Gene Names
UniProt Entry Name
IDHC_RAT
NCBI Summary for IDH1
catalyzes oxidative decarboxylation of citrate; cytosolic NADP+-dependent isozyme [RGD, Feb 2006]
UniProt Comments for IDH1
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: EC 1.1.1.42; Carbohydrate Metabolism - citrate (TCA) cycle; Oxidoreductase; Other Amino Acids Metabolism - glutathione
Cellular Component: mitochondrion; cytoplasm; peroxisome; cytosol
Molecular Function: protein homodimerization activity; magnesium ion binding; isocitrate dehydrogenase (NADP+) activity; NADP binding; NAD binding; receptor binding
Biological Process: glyoxylate cycle; isocitrate metabolic process; glutathione metabolic process; tricarboxylic acid cycle; response to steroid hormone stimulus; response to oxidative stress; response to organic cyclic substance; female gonad development; 2-oxoglutarate metabolic process
Research Articles on IDH1
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Products associated with IDH1 sirna
Pathways associated with IDH1 sirna
Diseases associated with IDH1 sirna
Organs/Tissues associated with IDH1 sirna
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