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Gene Symbol IDH2
Synonyms D2HGA2 | ICD-M | IDH | IDH-2 | IDHM | IDP | IDPM | mNADP-IDH
Gene Description IDH2, isocitrate dehydrogenase (NADP(+)) 2, is an enzyme that catalyzes the conversion of isocitrate to alpha-ketoglutarate (alpha-KG), and is involved in the tricarboxylic acid cycle (PMID: 28711227, PMID: 28980701). Mutations in IDH2 are associated with aberrant conversion of alpha-KG to 2-HG, which is an oncogenic metabolite, and are recurrent in acute myeloid leukemia and subtypes of glioma (PMID: 27621679, PMID: 19228619, PMID: 23999441).

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Molecular Profile Protein Effect Treatment Approaches
IDH2 A101P unknown
IDH2 A347T no effect - predicted
IDH2 R140Q IDH2 A347T
IDH2 A370T unknown
IDH2 A410S unknown
IDH2 A416fs unknown
IDH2 A416V unknown
IDH2 A47V unknown
IDH2 act mut gain of function IDH Inhibitor (Pan) IDH2 Inhibitor
IDH2 amp no effect
IDH2 C402Y unknown
IDH2 D76fs loss of function - predicted
IDH2 D83V unknown
IDH2 del loss of function
IDH2 E208* loss of function - predicted
IDH2 E226K unknown
IDH2 E268D unknown
IDH2 E343V no effect - predicted
IDH2 R140Q IDH2 E343V
IDH2 E345G no effect - predicted
IDH2 R140Q IDH2 E345G
IDH2 E68K unknown
IDH2 F394I loss of function - predicted
IDH2 F394V loss of function - predicted
IDH2 G137E unknown
IDH2 G144R unknown
IDH2 G145fs loss of function - predicted
IDH2 G145R unknown
IDH2 G171D unknown
IDH2 G190D unknown
IDH2 G201D unknown
IDH2 G260W unknown
IDH2 G421S unknown
IDH2 G450S no effect - predicted
IDH2 R140Q IDH2 G450S
IDH2 H348Q no effect - predicted
IDH2 R140Q IDH2 H348Q
IDH2 H358R unknown
IDH2 I139F unknown
IDH2 I228L unknown
IDH2 I319M no effect - predicted
IDH2 R140Q IDH2 I319M
IDH2 I441T unknown
IDH2 inact mut loss of function
IDH2 K130del unknown
IDH2 K133R unknown
IDH2 K280N unknown
IDH2 K48N unknown
IDH2 L143M unknown
IDH2 L449V no effect - predicted
IDH2 R140Q IDH2 L449V
IDH2 M248T unknown
IDH2 mutant unknown
CDKN2A del IDH2 mut
IDH2 N136S no effect - predicted
IDH2 N136S IDH2 R140Q
IDH2 over exp no effect
IDH2 P151H unknown
IDH2 P158L unknown
IDH2 P158T unknown
IDH2 P162S unknown
IDH2 Q316E no effect - predicted
IDH2 R140Q IDH2 Q316E
IDH2 Q322K unknown
IDH2 Q359H unknown
IDH2 R140G gain of function - predicted IDH Inhibitor (Pan) IDH2 Inhibitor
IDH2 R140K unknown
IDH2 R140L unknown IDH Inhibitor (Pan) IDH2 Inhibitor
IDH2 R140M unknown
IDH2 R140Q gain of function IDH Inhibitor (Pan) IDH2 Inhibitor
FLT3 exon 14 ins IDH2 R140Q
IDH1 R132C IDH1 D279N IDH2 R140Q
IDH1 R119P IDH1 R132L IDH2 R140Q
IDH1 R132L IDH2 R140Q
IDH1 R132C IDH2 R140Q
IDH1 R132H IDH2 R140Q
IDH2 R140Q IDH2 V351I
IDH2 R140Q IDH2 T352A
IDH2 R140Q IDH2 R353H
EML4 - ALK IDH2 R140Q
IDH2 R140S unknown
IDH2 R140W gain of function - predicted IDH Inhibitor (Pan) IDH2 Inhibitor
FLT3 Y599_D600insSTDNEYFYVDFREYEY IDH2 R140W
IDH2 R140X unknown IDH Inhibitor (Pan) IDH2 Inhibitor
IDH2 R159H unknown
IDH2 R172G gain of function IDH Inhibitor (Pan) IDH2 Inhibitor
IDH2 R172K gain of function IDH Inhibitor (Pan) IDH2 Inhibitor
IDH1 R132C IDH2 R172K
IDH2 R172M gain of function IDH Inhibitor (Pan) IDH2 Inhibitor
IDH2 R172S gain of function IDH Inhibitor (Pan) IDH2 Inhibitor
IDH2 R172T unknown
IDH2 R172W gain of function - predicted IDH Inhibitor (Pan) IDH2 Inhibitor
IDH2 R172X unknown IDH Inhibitor (Pan) IDH2 Inhibitor
IDH2 R353fs unknown
IDH2 R353H no effect - predicted
IDH2 R377C unknown
IDH2 S301L unknown
IDH2 T146fs loss of function - predicted
IDH2 T331M unknown
IDH2 T352A no effect - predicted
IDH2 T435M unknown
IDH2 V294M no effect - predicted
IDH2 V305M unknown
IDH2 V351I no effect - predicted
IDH2 W244* loss of function - predicted
IDH2 W375* unknown
IDH2 wild-type no effect