isocitrate dehydrogenase (NADP(+)) 2 | Isocitrate dehydrogenases | IUPHAR/BPS Guide to PHARMACOLOGY

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isocitrate dehydrogenase (NADP(+)) 2

Target not currently curated in GtoImmuPdb

Target id: 2885

Nomenclature: isocitrate dehydrogenase (NADP(+)) 2

Systematic Nomenclature: IDH2

Family: Isocitrate dehydrogenases

Annotation status:  image of a grey circle Awaiting annotation/under development. Please contact us if you can help with annotation.  » Email us

Gene and Protein Information
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human - 452 15q26.1 IDH2 isocitrate dehydrogenase (NADP(+)) 2
Mouse - 452 7 D3 Idh2 isocitrate dehydrogenase 2 (NADP+)
Rat - 452 1q31 Idh2 isocitrate dehydrogenase (NADP(+)) 2
Previous and Unofficial Names
IDH-2 | IDPm | isocitrate dehydrogenase 2 (NADP+) | isocitrate dehydrogenase 2 (NADP+), mitochondrial | isocitrate dehydrogenase 2 (NADP), mitochondrial | isocitrate dehydrogenase (NADP(+)) 2, mitochondrial
Database Links
ChEMBL Target
Ensembl Gene
Entrez Gene
Human Protein Atlas
RefSeq Nucleotide
RefSeq Protein

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Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Value Parameter Reference
enasidenib Hs Binding - - 1
vorasidenib Hs Inhibition - - 3
Description: Inhibits mutant IDH2.
Inhibitor Comments
Note that enasidenib is designed to inhibit mutant IDH2 isoforms.
Clinically-Relevant Mutations and Pathophysiology
Click column headers to sort
Type Species Amino acid change Nucleotide change Description Reference
Missense Human R172G, R172W, R172K, R172M, R172S 514A>G, 514A>T, 515G>A, 515G>T, 515G>C or T Mutations in IDH2 associated with cancers including glioma, acute myeloid leukemia, and myelodysplastic syndromes. 2,4-5
Clinically-Relevant Mutations and Pathophysiology Comments
Mutations generally involve missense mutations at the R140 and R172 residues of the protein. IDH2 mutations account for 5-10% of all IDH mutations in glioma.


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1. Cianchetta G, Delabarre B, Popovici-Muller J, Salituro FG, Saunders JO, Travins J, Yan S, Guo T, Zhang L. (2013) Therapeutically active compounds and their methods of use. Patent number: US20130190287 A1. Assignee: Agios Pharmaceuticals, Inc.. Priority date: 06/01/2012. Publication date: 25/07/2013.

2. Haferlach T, Nagata Y, Grossmann V, Okuno Y, Bacher U, Nagae G, Schnittger S, Sanada M, Kon A, Alpermann T et al.. (2014) Landscape of genetic lesions in 944 patients with myelodysplastic syndromes. Leukemia, 28 (2): 241-7. [PMID:24220272]

3. Konteatis Z, Artin E, Nicolay B, Straley K, Padyana AK, Jin L, Chen Y, Narayaraswamy R, Tong S, Wang F et al.. (2020) Vorasidenib (AG-881): A First-in-Class, Brain-Penetrant Dual Inhibitor of Mutant IDH1 and 2 for Treatment of Glioma. ACS Med Chem Lett, 11 (2): 101-107. DOI: 10.1021/acsmedchemlett.9b00509 [PMID:32071674]

4. Walter MJ, Shen D, Shao J, Ding L, White BS, Kandoth C, Miller CA, Niu B, McLellan MD, Dees ND et al.. (2013) Clonal diversity of recurrently mutated genes in myelodysplastic syndromes. Leukemia, 27 (6): 1275-82. [PMID:23443460]

5. Yang H, Ye D, Guan KL, Xiong Y. (2012) IDH1 and IDH2 mutations in tumorigenesis: mechanistic insights and clinical perspectives. Clin. Cancer Res., 18 (20): 5562-71. [PMID:23071358]

How to cite this page Isocitrate dehydrogenases: isocitrate dehydrogenase (NADP(+)) 2. Last modified on 11/02/2020. Accessed on 10/04/2020. IUPHAR/BPS Guide to PHARMACOLOGY,