Location [1]
Chromatin remodeling/DNA methylation, Metabolic signaling
Protein [2]
Isocitrate dehydrogenase [NADP], mitochondrial
Synonyms [1]

IDH2 (isocitrate dehydrogenase 2 (NADP+), mitochondrial), encodes for the isocitrate dehydrogenase [NADP], mitochondrial protein, an epigenetic modifier. IDH2 is frequently mutated in glioma and acute myeloid leukemia (PMID: 23071358).

IDH2 is altered in 1.30% of all cancers with leukemia, breast carcinoma, colorectal adenocarcinoma, malignant glioma, and non-small cell lung carcinoma having the greatest prevalence of alterations [3].

IDH2 GENIE Cases - Top Diseases

The most common alterations in IDH2 are IDH2 Mutation (1.35%), IDH2 Codon 140 Missense (0.41%), IDH2 R140Q (0.38%), IDH2 Codon 172 Missense (0.34%), and IDH2 Amplification (0.20%) [3].

IDH2 GENIE Cases - Top Alterations

Biomarker-Directed Therapies

Significance of IDH2 in Diseases

Acute Myeloid Leukemia +

Glioma +

Malignant Solid Tumor +

Myelodysplastic Syndromes +

Cholangiocarcinoma +

Chronic Myelomonocytic Leukemia +

Oligodendroglioma +

WHO Grade II Glioma +

Malignant Glioma +

Astrocytoma +

Clear Cell Renal Cell Carcinoma +

Multiple Myeloma +

Angioimmunoblastic T-Cell Lymphoma +

Oligoastrocytoma +

Leukemia +

WHO Grade III Glioma +

Myelofibrosis +

Lymphoma +

Anaplastic Astrocytoma +

Glioblastoma +

Meningioma +

Breast Carcinoma +

Non-Small Cell Lung Carcinoma +

Renal Cell Carcinoma +

Gastrointestinal Stromal Tumor +

Acute Bilineal Leukemia +

Acute Biphenotypic Leukemia +

Diffuse Astrocytoma +

Ependymoma +

Gallbladder Carcinoma +

Mesothelioma +

Papillary Renal Cell Carcinoma +

Peritoneal Mesothelioma +

Plasma Cell Leukemia +

Pleural Mesothelioma +

Primary Central Nervous System Lymphoma +

Uveal Melanoma +


1. Hart R and Prlic A. Universal Transcript Archive Repository. Version uta_20170629. San Francisco CA: Github;2015. https://github.com/biocommons/uta

2. The UniProt Consortium. UniProt: a worldwide hub of protein knowledge. Nucleic Acids Research. 2019;47:D506-D515.

3. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 4. This dataset does not represent the totality of the genetic landscape; see paper for more information.

4. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.