Recombinant Human Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial (IDH3G)

Uniprot Id

P51553

Target Species

Human

Target Name

IDH3G

Target Full Name

Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial

Target Function

Regulatory subunit which plays a role in the allosteric regulation of the enzyme catalyzing the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers.

Target Subcellular Location

Mitochondrion.

Target Protein Families

Isocitrate and isopropylmalate dehydrogenases family

Target Research Area

Signal Transduction

Target Synonyms

H IDHG; IDH gamma; IDH3G; IDH3G_HUMAN; Isocitrate dehydrogenase [NAD] subunit gamma; Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial; Isocitrate dehydrogenase 3 (NAD+) gamma; Isocitric dehydrogenase; Isocitric dehydrogenase subunit gamma; mitochondrial; NAD (H) specific isocitrate dehydrogenase gamma subunit; NAD(+) specific ICDH; NAD(+)-specific ICDH subunit gamma; NAD+ specific ICDH; OTTHUMP00000025984; OTTHUMP00000025985; OTTHUMP00000025987; OTTHUMP00000025988; OTTHUMP00000214764

Target 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.