Overview

  • Product name

    Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody
    See all Pyruvate Dehydrogenase E1-alpha subunit primary antibodies
  • Description

    Rabbit polyclonal to Pyruvate Dehydrogenase E1-alpha subunit
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-P, IHC - Wholemountmore details
  • Species reactivity

    Reacts with: Zebrafish
  • Immunogen

    Recombinant fragment within Zebrafish Pyruvate Dehydrogenase E1-alpha subunit aa 62-348. The exact sequence is proprietary.
    Database link: Q6P948

  • Positive control

    • Zebrafish embryo; zebrafish eye whole cell lysate; zebrafish kidney tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab210533 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 1/500 - 1/3000. Predicted molecular weight: 44 kDa.
IHC-P 1/100 - 1/1000.

Suggested antigen retrieval using 1mM EDTA-NaOH (pH8.0) 95ºC,1Hr

IHC - Wholemount 1/100 - 1/500.

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).
  • Tissue specificity

    Ubiquitous.
  • Involvement in disease

    Defects in PDHA1 are a cause of pyruvate decarboxylase E1 component deficiency (PDHE1 deficiency) [MIM:312170]. PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (X-LS).
    Defects in PDHA1 are the cause of X-linked Leigh syndrome (X-LS) [MIM:308930]. X-LS is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord. The lesions are areas of demyelination, gliosis, necrosis, spongiosis, or capillary proliferation. Clinical symptoms depend on which areas of the central nervous system are involved. The most common underlying cause is a defect in oxidative phosphorylation. LS may be a feature of a deficiency of any of the mitochondrial respiratory chain complexes.
  • Cellular localization

    Mitochondrion matrix.
  • Information by UniProt
  • Database links

  • Alternative names

    • ODPA_HUMAN antibody
    • PDH antibody
    • PDHA antibody
    • PDHA1 antibody
    • PDHCE1A antibody
    • PDHE1 A type I antibody
    • PDHE1-A type I antibody
    • PHE1A antibody
    • Pyruvate Dehydrogenase (lipoamide) alpha 1 antibody
    • Pyruvate dehydrogenase complex, E1 alpha polypeptide 1 antibody
    • Pyruvate Dehydrogenase E1 alpha antibody
    • Pyruvate dehydrogenase E1 component subunit alpha antibody
    • Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial antibody
    see all

Images

  • Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody (ab210533) at 1/1000 dilution + zebrafish eye whole cell lysate at 30 µg

    Predicted band size: 44 kDa



    10% SDS-PAGE

  • Immunohistochemical analysis of paraffin-embedded Zebrafish kidney tissue labeling Pyruvate Dehydrogenase E1-alpha with ab210533 at 1/300 dilution.

  • Immunohistochemical analysis (wholemount) of zebrafish embryo labeling Pyruvate Dehydrogenase E1-alpha with ab210533 at 1/200 dilution.

References

ab210533 has not yet been referenced specifically in any publications.

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