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

isocitrate dehydrogenase (NADP(+)) 2, mitochondrial

Target id: 2885

Nomenclature: isocitrate dehydrogenase (NADP(+)) 2, mitochondrial

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

   GtoImmuPdb view: OFF :     Currently no data for isocitrate dehydrogenase (NADP(+)) 2, mitochondrial in GtoImmuPdb

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

Download all structure-activity data for this target as a CSV file

Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Affinity Units Reference
enasidenib Hs Binding - - 1
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
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.


Show »

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. 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]

4. 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, mitochondrial. Last modified on 22/06/2016. Accessed on 20/09/2018. IUPHAR/BPS Guide to PHARMACOLOGY,