Application
Immunofluorescence staining of formalin-fixed Hep G2 cells showing mitochondria localization.Application
Western blot analysis of E3BP expression in Hep G2 whole cell lysate.| Product Name | E3BP Antibody (H-130) |
|---|---|
| Antibody Type | Primary Antibodies |
| Modification Notes | The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial matrix enzyme complex that functions as the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the irreversible conversion of pyruvate into acetyl-CoA. E3BP (E3-binding protein), also known as PDHX (Pyruvate dehydrogenase protein X component) and Lipoyl-containing pyruvate dehydrogenase complex component X, is a 501 amino acid mitochondrial protein that is required for anchoring E3 to the E2 core of the PDH complex, an event that is essential for a functional PDH complex. Defects in the gene encoding E3BP result in pyruvate dehydrogenase E3-binding protein deficiency, which is similar to PDH deficiency and Leigh syndrome in clinical presentation. Symptoms of E3BP deficiency can include lactic acidosis, delayed development, seizures, diplegia, cerebellar ataxia, optic atrophy, facial dysmorphism and episodic weakness. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of E3BP of human and, to a lesser extent, mouse and rat origin by WB, IP, IF and ELISA | |
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 151-280 mapping within an internal region of E3BP of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 151-280 mapping within an internal region of E3BP of human origin |
| Entrez Gene | 8050 |
|---|
Application
Immunofluorescence staining of formalin-fixed Hep G2 cells showing mitochondria localization.
Application
Western blot analysis of E3BP expression in Hep G2 whole cell lysate.| Application Notes | recommended for detection of E3BP of human and, to a lesser extent, mouse and rat origin by WB, IP, IF and ELISA |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
