产品名称Anti-Pyruvate Dehydrogenase E2抗体[15D3G9C11]
参阅全部 Pyruvate Dehydrogenase E2 一抗
描述小鼠单克隆抗体[15D3G9C11] to Pyruvate Dehydrogenase E2
经测试应用适用于: WB, ICC/IF, Flow Cyt, IHC-P, In-Cell ELISAmore details
种属反应性与反应: Cow, Human
Porcine Pyruvate Dehydrogenase E2 protein.
- Isolated mitochondria from Human heart; Normal Human embryonic lung fibroblasts (strain MRC5); Human cerebellum tissue; HL60 cells.
存放说明Shipped at 4°C. Store at +4°C. Do Not Freeze.
存储溶液Preservative: 0.02% Sodium azide
Constituent: HEPES buffered saline
Concentration information loading...
纯化说明ab110332 was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
- Pathways and Processes
- Metabolic signaling pathways
- Energy transfer pathways
- Energy Metabolism
Our Abpromise guarantee covers the use of ab110332 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 0.5 µg/ml. Predicted molecular weight: 69 kDa.|
|ICC/IF||Use a concentration of 0.2 - 0.5 µg/ml. (heat-induced antigen-retrieval improves signal).|
|Flow Cyt||Use a concentration of 1 µg/ml.
ab170190 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.
|IHC-P||1/100. Perform heat mediated antigen retrieval via the pressure cooker method before commencing with IHC staining protocol.|
|In-Cell ELISA||Use a concentration of 1 µg/ml.|
功能The 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).
疾病相关Note=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.
序列相似性Belongs to the 2-oxoacid dehydrogenase family.
Contains 2 lipoyl-binding domains.
- Information by UniProt
- 70 kDa mitochondrial autoantigen of primary biliary cirrhosis antibody
- Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex antibody
- Dihydrolipoamide S Acetyltransferase antibody
Anti-Pyruvate Dehydrogenase E2 antibody [15D3G9C11] (ab110332) at 0.5 µg/ml + Isolated mitochondria from Human heart at 5 µg
Predicted band size: 69 kDa
Immunocytochemistry analysis using ab110332 at 1µg/ml staining Pyruvate Dehydrogenase E2 in cultured, normal Human embryonic lung fibroblasts (strain MRC5) and an AlexaFluor® 488 goat anti-mouse IgG1 secondary antibody (2 ug/ml).
Immunohistological analysis using ab110332 at 1/100 dilution staining Pyruvate Dehydrogenase E2 in Human cerebellum tissue (Formalin-fixed, Paraffin-embedded).
Flow cytometric analysis using ab110332 at 1µg/ml staining Pyruvate Dehydrogenase E2 in HL60 cells (blue). Isotype control antibody (red).
This product has been referenced in:
- Matsuda S et al. Nuclear pyruvate kinase M2 complex serves as a transcriptional coactivator of arylhydrocarbon receptor. Nucleic Acids Res 44:636-47 (2016). Read more (PubMed: 26405201) »
- Sloan JL et al. Exome sequencing identifies ACSF3 as a cause of combined malonic and methylmalonic aciduria. Nat Genet : (2011). WB ; Human . Read more (PubMed: 21841779) »