Anti-Pyruvate Dehydrogenase E2 antibody [15D3G9C11] (ab110332)

Overview

  • Product nameAnti-Pyruvate Dehydrogenase E2 antibody [15D3G9C11]
    See all Pyruvate Dehydrogenase E2 primary antibodies
  • Description
    Mouse monoclonal [15D3G9C11] to Pyruvate Dehydrogenase E2
  • Tested applicationsSuitable for: WB, ICC/IF, Flow Cyt, IHC-P, In-Cell ELISAmore details
  • Species reactivity
    Reacts with: Cow, Human
  • Immunogen

    Porcine Pyruvate Dehydrogenase E2 protein.

  • Positive control
    • Isolated mitochondria from Human heart; Normal Human embryonic lung fibroblasts (strain MRC5); Human cerebellum tissue; HL60 cells.
  • General notes

    Product was previously marketed under the MitoSciences sub-brand.

Properties

Applications

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.

Application Abreviews Notes
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.

Target

  • 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
  • Database links
  • Alternative names
    • 70 kDa mitochondrial autoantigen of primary biliary cirrhosis antibody
    • Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex antibody
    • Dihydrolipoamide S Acetyltransferase antibody
    • Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex antibody
    • dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial antibody
    • DLAT antibody
    • DLTA antibody
    • E2 antibody
    • E2 component of pyruvate dehydrogenase complex antibody
    • M2 antigen complex 70 kDa subunit antibody
    • mitochondrial antibody
    • ODP2_HUMAN antibody
    • PBC antibody
    • PDC E2 antibody
    • PDC-E2 antibody
    • PDCE2 antibody
    • Pyruvate dehydrogenase complex component E2 antibody
    • Pyruvate dehydrogenase complex E2 subunit antibody
    see all

Anti-Pyruvate Dehydrogenase E2 antibody [15D3G9C11] images

  • 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).

References for Anti-Pyruvate Dehydrogenase E2 antibody [15D3G9C11] (ab110332)

This product has been referenced in:
  • 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) »
  • Feng D  et al. Down-regulation of mitochondrial acyl carrier protein in mammalian cells compromises protein lipoylation and respiratory complex I and results in cell death. J Biol Chem 284:11436-45 (2009). Read more (PubMed: 19221180) »

See all 6 Publications for this product

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