IDH2

Isocitrate dehydrogenase [NADP], mitochondrial is an enzyme that in humans is encoded by the IDH2 gene.

IDH2
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesIDH2, D2HGA2, ICD-M, IDH, IDHM, IDP, IDPM, mNADP-IDH, isocitrate dehydrogenase (NADP(+)) 2, mitochondrial, isocitrate dehydrogenase (NADP(+)) 2, IDH-2
External IDsOMIM: 147650 MGI: 96414 HomoloGene: 37590 GeneCards: IDH2
Orthologs
SpeciesHumanMouse
Entrez

3418

269951

Ensembl

ENSG00000182054

ENSMUSG00000030541

UniProt

P48735

P54071

RefSeq (mRNA)

NM_002168
NM_001289910
NM_001290114

NM_173011

RefSeq (protein)

NP_001276839
NP_001277043
NP_002159

NP_766599

Location (UCSC)Chr 15: 90.08 – 90.1 MbChr 7: 79.74 – 79.77 Mb
PubMed search
Wikidata
View/Edit HumanView/Edit Mouse

Isocitrate dehydrogenases are enzymes that 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. Each NADP(+)-dependent isozyme is a homodimer. The protein encoded by the IDH2 gene is the NADP(+)-dependent isocitrate dehydrogenase found in the mitochondria. It plays a role in intermediary metabolism and energy production. This protein may tightly associate or interact with the pyruvate dehydrogenase complex. Somatic mosaic mutations of this gene have also been found associated to Ollier disease and Maffucci syndrome.

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