Overview

  • Product name
    Anti-Pyruvate Dehydrogenase E2 antibody
    See all Pyruvate Dehydrogenase E2 primary antibodies
  • Description
    Rabbit polyclonal to Pyruvate Dehydrogenase E2
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Recombinant fragment corresponding to a region within amino acids 310-548 of Human Pyruvate Dehydrogenase E2 (UniProt ID: P10515).

  • Positive control
    • 293T, A431, HeLa and HepG2 whole cell lysates

Properties

Applications

Our Abpromise guarantee covers the use of ab126224 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 1/5000 - 1/20000. Predicted molecular weight: 69 kDa.

Target

  • Function
    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).
  • Involvement in disease
    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.
  • Sequence similarities
    Belongs to the 2-oxoacid dehydrogenase family.
    Contains 2 lipoyl-binding domains.
  • Cellular localization
    Mitochondrion 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

Images

  • All lanes : Anti-Pyruvate Dehydrogenase E2 antibody (ab126224) at 1/10000 dilution

    Lane 1 : 293T whole cell lysate
    Lane 2 : A431 whole cell lysate
    Lane 3 : HeLa whole cell lysate
    Lane 4 : HepG2 whole cell lysate

    Lysates/proteins at 30 µg per lane.


    Predicted band size : 69 kDa

References

This product has been referenced in:
  • Osinalde N  et al. Changes in Gab2 phosphorylation and interaction partners in response to interleukin (IL)-2 stimulation in T-lymphocytes. Sci Rep 6:23530 (2016). WB . Read more (PubMed: 27025927) »

See 1 Publication for this product

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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