IDH2 (isocitrate dehydrogenase 2 (NADP+), mitochondrial), also designated NADP+-specific ICDH; isocitrate dehydrogenase, mitochondrial; and oxalosuccinate decarboxylase, is a 452 amino acid enzyme encoded by the human gene IDH2. IDH2 belongs to the isocitrate and isopropylmalate dehydrogenases family and contains two nucleotide binding regions. IDH2 is involved in the reduction of NADP+ to NADPH and maintains the supply of glutathione (GSH) in mitochondria. It is believed to play a role in intermediary metabolism and energy production. IDH2 also tightly associates with the pyruvate dehydrogenase complex. IDH2 is found in the mitochondrion as a homodimer and can bind one magnesium or manganese ion per subunit.
Function:
Plays a role in intermediary metabolism and energy production. It may tightly associate or interact with the pyruvate dehydrogenase complex.
Subunit:
Homodimer.
Subcellular Location:
Cytoplasm. Peroxisome.
DISEASE:
Defects in IDH1 are involved in the development of glioma (GLM) [MIM:137800]. Gliomas are central nervous system neoplasms derived from glial cells and comprise astrocytomas, glioblastoma multiforme, oligodendrogliomas, and ependymomas. Note=Mutations affecting Arg-132 are tissue-specific, and suggest that this residue plays a unique role in the development of high-grade gliomas. Mutations of Arg-132 to Cys, His, Leu or Ser abolish magnesium binding and abolish the conversion of isocitrate to alpha-ketoglutarate. Instead, alpha-ketoglutarate is converted to R(-)-2-hydroxyglutarate. Elevated levels of R(-)-2-hydroxyglutarate are correlated with an elevated risk of malignant brain tumors.
Similarity:
Belongs to the isocitrate and isopropylmalate dehydrogenases family.
SWISS:
P48735
Gene ID:
3418
Database links:
Entrez Gene: 3418 Human
Entrez Gene: 269951 Mouse
Entrez Gene: 361596 Rat
Omim: 147650 Human
SwissProt: P48735 Human
SwissProt: P54071 Mouse
SwissProt: P56574 Rat
Unigene: 596461 Human
Unigene: 246432 Mouse
Unigene: 290925 Mouse
Unigene: 3490 Rat
IDH2又称异柠檬酸脱氢酶，他有有两种类型的酶：以NAD为辅酶的酶（EC1．1．1．41）和NADP的酶（EC1．1．1．42），两者催化同一反应。在胞浆中以NADP为辅酶，在线粒体内膜以NAD为辅酶。
IDH2在三羧酸循环中正常起作用的是NAD为辅酶的酶，他是提供为ADP激活的变构酶。
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Paraformaldehyde-fixed, paraffin embedded (Rat liver); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (IDH2) Polyclonal Antibody, Unconjugated (SL3947R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Tissue/cell: human lung carcinoma; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,SLC0005) at 37℃ for 20 min;
Incubation: Anti-IDH2 Polyclonal Antibody, Unconjugated(SL3947R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(SLC0010) staining
Tissue/cell: human lung carcinoma;4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Blocking buffer (normal goat serum,SLC0005) at 37℃ for 20 min;
Incubation: Anti-IDH2 Polyclonal Antibody, Unconjugated(SL3947R) 1:200, overnight at 4°C; The secondary antibody was Goat Anti-Rabbit IgG, Cy3 conjugated(SL0295G-Cy3)used at 1:200 dilution for 40 minutes at 37°C. DAPI(5ug/ml,blue,SLC0033) was used to stain the cell nuclei
U-937 cells were fixed with 4% PFA for 10min at room temperature,permeabilized with 20% PBST for 20 min at room temperature, and incubated in 5% BSA blocking buffer for 30 min at room temperature. Cells were then stained with IDH2 Antibody(SL3947R)at 1:500 dilution in blocking buffer and incubated for 30 min at room temperature, washed twice with 2%BSA in PBS, followed by secondary antibody incubation for 40 min at room temperature. Acquisitions of 20,000 events were performed. Cells stained with primary antibody (green), and isotype control (orange).
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