Background: | Isocitrate dehydrogenases catalyze the oxidative decarboxylation of isocitrate to 2-oxoglutarate. These enzymes belong to two distinct subclasses, one of which utilizes NAD(+) as the electron acceptor and the other NADP(+). Five isocitrate dehydrogenases have been reported: three NAD(+)-dependent isocitrate dehydrogenases, which localize to the mitochondrial matrix, and two NADP(+)-dependent isocitrate dehydrogenases, one of which is mitochondrial and the other predominantly cytosolic. NAD(+)-dependent isocitrate dehydrogenases catalyze the allosterically regulated rate-limiting step of the tricarboxylic acid cycle. Each isozyme is a heterotetramer that is composed of two alpha subunits, one beta subunit, and one gamma subunit. The protein encoded by this gene is the gamma subunit of one isozyme of NAD(+)-dependent isocitrate dehydrogenase. This gene is a candidate gene for periventricular heterotopia. Several alternatively spliced transcript variants of this gene have been described, but only some of their full length natures have been determined. |
UniProt Protein Function: | IDH3G: a enzyme that that catalyzes the oxidative decarboxylation of isocitrate to alpha-ketoglutarate (alpha-KG), requiring nicotinamide adenine dinucleotide (NAD) as a cofactor. The holoenzyme is a heterooligomer of subunits alpha, beta, and gamma in the apparent ratio of 2:1:1. IDH3 is a mitochondrial enzyme that functions in the Krebs cycle, which generates substrates for cellular respiration. Alpha-KG can exit the mitochondria and enter other cellular compartments where it is a cofactor for the dioxygenases that hydroxylate the transcription factor HIF and trigger its degradation by VHL. Since HIF turns on oncogenic pathways, IDH3 may have tumor suppressor activity. Two isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Carbohydrate Metabolism - citrate (TCA) cycle; Oxidoreductase; EC 1.1.1.41; Mitochondrial Chromosomal Location of Human Ortholog: Xq28 |
NCBI Summary: | Isocitrate dehydrogenases catalyze the oxidative decarboxylation of isocitrate to 2-oxoglutarate. These enzymes belong to two distinct subclasses, one of which utilizes NAD(+) as the electron acceptor and the other NADP(+). Five isocitrate dehydrogenases have been reported: three NAD(+)-dependent isocitrate dehydrogenases, which localize to the mitochondrial matrix, and two NADP(+)-dependent isocitrate dehydrogenases, one of which is mitochondrial and the other predominantly cytosolic. NAD(+)-dependent isocitrate dehydrogenases catalyze the allosterically regulated rate-limiting step of the tricarboxylic acid cycle. Each isozyme is a heterotetramer that is composed of two alpha subunits, one beta subunit, and one gamma subunit. The protein encoded by this gene is the gamma subunit of one isozyme of NAD(+)-dependent isocitrate dehydrogenase. This gene is a candidate gene for periventricular heterotopia. Several alternatively spliced transcript variants of this gene have been described, but only some of their full length natures have been determined. [provided by RefSeq, Jul 2008] |
UniProt Code: | P51553 |
NCBI GenInfo Identifier: | 1708404 |
NCBI Gene ID: | 3421 |
NCBI Accession: | P51553.1 |
UniProt Secondary Accession: | P51553,Q9BUU5, E9PDD5, |
UniProt Related Accession: | P51553 |
Molecular Weight: | 41,468 Da |
NCBI Full Name: | Isocitrate dehydrogenase |
NCBI Synonym Full Names: | isocitrate dehydrogenase 3 (NAD(+)) gamma |
NCBI Official Symbol: | IDH3G |
NCBI Official Synonym Symbols: | H-IDHG |
NCBI Protein Information: | isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial |
UniProt Protein Name: | Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial |
UniProt Synonym Protein Names: | Isocitric dehydrogenase subunit gamma; NAD(+)-specific ICDH subunit gamma |
Protein Family: | Isocitrate dehydrogenase |
UniProt Gene Name: | IDH3G |
UniProt Entry Name: | IDH3G_HUMAN |