Product nameAnti-Pyruvate dehydrogenase E2/E3bp antibody [13G2AE2BH5]
DescriptionMouse monoclonal [13G2AE2BH5] to Pyruvate dehydrogenase E2/E3bp
Tested applicationsSuitable for: WB, ICC/IF, Flow Cyt, IHC-Pmore details
Species reactivityReacts with: Mouse, Rat, Cow, Human
Porcine Pyruvate dehydrogenase E2/E3bp.
- Isolated mitochondria from Human, Bovine, Rat and Mouse heart and HepG2 lysate; cultured, normal Human embryonic lung fibroblasts (strain MRC5); Human cerebellum tissue; HL60 cells.
Storage instructionsShipped at 4°C. Store at +4°C. Do Not Freeze.
Storage bufferPreservative: 0.02% Sodium azide
Constituent: HEPES buffered saline
Concentration information loading...
Purification notesab110333 was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
Light chain typekappa
- Pathways and Processes
- Metabolic signaling pathways
- Energy transfer pathways
- Energy Metabolism
Our Abpromise guarantee covers the use of ab110333 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 1 µg/ml. Predicted molecular weight: 54 , 69 kDa.|
|ICC/IF||Use a concentration of 1 µg/ml. (heat-induced antigen-retrieval improves signal)|
|Flow Cyt||Use a concentration of 1 mg/ml.
ab170191 - Mouse monoclonal IgG2a, is suitable for use as an isotype control with this antibody.
|IHC-P||1/1000. Perform heat mediated antigen retrieval via the pressure cooker method before commencing with IHC staining protocol.|
FunctionThe pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3).
Involvement in diseaseNote=Primary biliary cirrhosis is a chronic, progressive cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients' serum. It manifests with inflammatory obliteration of intra-hepatic bile duct, leading to liver cell damage and cirrhosis. Patients with primary biliary cirrhosis show autoantibodies against the E2 component of pyruvate dehydrogenase complex.
Defects in DLAT are the cause of pyruvate dehydrogenase E2 deficiency (PDHE2 deficiency) [MIM:245348]; also known as lactic acidemia due to defect of E2 lipoyl transacetylase of the pyruvate dehydrogenase complex. Pyruvate dehydrogenase (PDH) deficiency is a major cause of primary lactic acidosis and neurological dysfunction in infancy and early childhood. In this form of PDH deficiency episodic dystonia is the major neurological manifestation, with other more common features of pyruvate dehydrogenase deficiency, such as hypotonia and ataxia, being less prominent.
Sequence similaritiesBelongs to the 2-oxoacid dehydrogenase family.
Contains 2 lipoyl-binding domains.
Cellular localizationMitochondrion matrix.
- Information by UniProt
- 70 kDa mitochondrial autoantigen of primary biliary cirrhosis antibody
- AI481367 antibody
- BOS_15094 antibody
All lanes : Anti-Pyruvate dehydrogenase E2/E3bp antibody [13G2AE2BH5] (ab110333) at 1 µg/ml
Lane 1 : Isolated mitochondria from Human heart at 5 µg
Lane 2 : Isolated mitochondria from Bovine heart at 1 µg
Lane 3 : Isolated mitochondria from Rat heart at 10 µg
Lane 4 : Isolated mitochondria from Mouse heart at 10 µg
Lane 5 : HepG2 cell lysate at 20 µg
Predicted band size: 54 , 69 kDa
ab110333 staining Pyruvate dehydrogenase E2/E3bp in Human HUVEC by ICC/IF (Immunocytochemistry/immunofluorescence). Cells were fixed with paraformaldehyde, permeabilized with 0.1% Triton X-100 pH 7.4 for 5 minutes and blocked with 5% BSA for 20 minutes at room temperature. Samples were incubated with primary antibody (1/1000 in PBS) for 1 hour. A CF568-conjugated Goat anti-mouse IgG polyclonal (1/500) was used as the secondary antibody.
Immunocytochemistry analysis using ab110333 at 1µg/ml staining Pyruvate dehydrogenase E2/E3bp in cultured, normal Human embryonic lung fibroblasts and an AlexaFluor® 488 goat anti-mouse IgG2a secondary antibody (2 ug/ml).
Immunohistological analysis using ab110333 at 1/1000 dilution staining Pyruvate dehydrogenase E2/E3bp in Human cerebellum tissue (Formalin-fixed, Paraffin-embedded).
Note: Immunoactivity is most intense in neuronal cell bodies, most notably in the large Purkinje cells.
Flow cytometric analysis using ab110333 at 1µg/ml staining Pyruvate dehydrogenase E2/E3b in HL60 cells (blue). Isotype control antibody (red).
This product has been referenced in:
- Gonzalez-Hurtado E et al. Fatty acid oxidation is required for active and quiescent brown adipose tissue maintenance and thermogenic programing. Mol Metab 7:45-56 (2018). WB ; Mouse . Read more (PubMed: 29175051) »
- McLelland GL et al. Mfn2 ubiquitination by PINK1/parkin gates the p97-dependent release of ER from mitochondria to drive mitophagy. Elife 7:N/A (2018). Read more (PubMed: 29676259) »