IDH1 Back

isocitrate dehydrogenase 1 (NADP+), soluble

External References:      Wikipedia GeneCards HUGO COSMIC Google Scholar

NCBI Description of IDH1

Isocitrate dehydrogenases catalyze the oxidative decarboxylation of isocitrate to 2-oxoglutarate. These enzymes belong to two distinct subclasses, one of which utilizes NAD(+) as the electron acceptor and the other NADP(+). Five isocitrate dehydrogenases have been reported: three NAD(+)-dependent isocitrate dehydrogenases, which localize to the mitochondrial matrix, and two NADP(+)-dependent isocitrate dehydrogenases, one of which is mitochondrial and the other predominantly cytosolic. Each NADP(+)-dependent isozyme is a homodimer. The protein encoded by this gene is the NADP(+)-dependent isocitrate dehydrogenase found in the cytoplasm and peroxisomes. It contains the PTS-1 peroxisomal targeting signal sequence. The presence of this enzyme in peroxisomes suggests roles in the regeneration of NADPH for intraperoxisomal reductions, such as the conversion of 2, 4-dienoyl-CoAs to 3-enoyl-CoAs, as well as in peroxisomal reactions that consume 2-oxoglutarate, namely the alpha-hydroxylation of phytanic acid. The cytoplasmic enzyme serves a significant role in cytoplasmic NADPH production.

Community Annotation of IDH1 Add / Edit IDH1: Annotations

No community annotations yet for IDH1.
Sort mutations by: Tumor type  Mutation type  Position  
Straightedge cursor Expand

Figure notes

• "Mouse over" a mutation to see details.
• Missense green saturation indicates evolutionary conservation of the mutated positions.
• Red hashes in protein strip are splice sites.
• Blue-white-red bars are log2 copy ratio distributions (–1 to +1) from Zack et al. (2013).


IDH1 is highly significantly mutated in
Acute myeloid leukemia
19 patients (9%)
Glioblastoma multiforme
15 patients (5%)
IDH1 is significantly mutated in
Multiple myeloma
3 patients (1%)
combined cohort
67 patients (1%)
IDH1 is near significance in
Chronic lymphocytic leukemia
2 patients (1%)

Click on a tumor type to see its full list of significant genes.

Data details

Mutation list for IDH1