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Gene | IDH1 |
Variant | R132H |
Impact List | missense |
Protein Effect | gain of function |
Gene Variant Descriptions | IDH1 R132H lies within the active site of the Idh1 protein (PMID: 19228619). R132H results in decreased conversion of isocitrate to alpha-ketoglutarate by Idh1, but also confers a gain of function to Idh1, as indicated by increased conversion of alpha-ketoglutarate to the onco-metabolite 2HG (R(-)-2-hydroxyglutarate) in cell culture and in vitro assays, and is associated with increased 2HG levels in patient samples (PMID: 19935646, PMID: 23731180). |
Associated Drug Resistance | |
Category Variants Paths |
IDH1 mutant IDH1 act mut IDH1 R132H IDH1 mutant IDH1 R132X IDH1 R132H |
Transcript | NM_005896.4 |
gDNA | chr2:g.208248388C>T |
cDNA | c.395G>A |
Protein | p.R132H |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_001282386.1 | chr2:g.208248388C>T | c.395G>A | p.R132H | RefSeq | GRCh38/hg38 |
NM_001282386 | chr2:g.208248388C>T | c.395G>A | p.R132H | RefSeq | GRCh38/hg38 |
NM_001282387.1 | chr2:g.208248388C>T | c.395G>A | p.R132H | RefSeq | GRCh38/hg38 |
NM_001282387.1 | chr2:g.208248388C>T | c.395G>A | p.R132H | RefSeq | GRCh38/hg38 |
NM_001282386.1 | chr2:g.208248388C>T | c.395G>A | p.R132H | RefSeq | GRCh38/hg38 |
NM_005896.4 | chr2:g.208248388C>T | c.395G>A | p.R132H | RefSeq | GRCh38/hg38 |
NM_005896 | chr2:g.208248388C>T | c.395G>A | p.R132H | RefSeq | GRCh38/hg38 |
NM_001282387 | chr2:g.208248388C>T | c.395G>A | p.R132H | RefSeq | GRCh38/hg38 |
NM_005896.3 | chr2:g.208248388C>T | c.395G>A | p.R132H | RefSeq | GRCh38/hg38 |
Molecular Profile | Protein Effect | Treatment Approaches |
---|---|---|
IDH1 R132H | gain of function | IDH Inhibitor (Pan) IDH1 Inhibitor IDH1 R132H peptide vaccine PEPIDH1M vaccine |
FLT3 D835Y IDH1 R132H | ||
FLT3 exon 14 ins IDH1 R132H | ||
IDH1 R132H IDH1 D279N | ||
IDH1 R132H IDH1 S280F | ||
IDH1 R132H IDH1 H315D | ||
IDH1 R132H IDH2 R140Q | ||
IDH1 R132H IDH1 T313I | ||
IDH1 R132H IDH1 A410T | ||
IDH1 R132H IDH1 L409V | ||
CDKN2A del IDH1 R132H |