Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody [9H9AF5] (HRP) (ab197956)

Overview

  • Product name

    Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody [9H9AF5] (HRP)
    See all Pyruvate Dehydrogenase E1-alpha subunit primary antibodies
  • Description

    Mouse monoclonal [9H9AF5] to Pyruvate Dehydrogenase E1-alpha subunit (HRP)
  • Host species

    Mouse
  • Conjugation

    HRP
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Cow
  • Immunogen

    Recombinant full length protein corresponding to Human Pyruvate Dehydrogenase E1-alpha subunit.

  • Positive control

    • WB: Human heart mitochondrial lysate and HepG2 whole cell lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab197956 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 1/3000. Detects a band of approximately 43 kDa (predicted molecular weight: 43 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).
  • 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

  • All lanes : Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody [9H9AF5] (HRP) (ab197956) at 1/3000 dilution

    Lane 1 : Human heart tissue lysate - mitochondrial extract (ab110337) at 10 µg
    Lane 2 : HepG2 (Human hepatocellular liver carcinoma cell line) Whole Cell Lysate at 20 µg

    Developed using the ECL technique.

    Performed under reducing conditions.

    Predicted band size: 43 kDa
    Observed band size: 43 kDa


    Exposure time: 8 minutes


    This blot was produced using a 4-12% Bis-tris gel under the MOPS buffer system. The gel was run at 200V for 50 minutes before being transferred onto a Nitrocellulose membrane at 30V for 70 minutes. The membrane was then blocked for an hour using 3% milk before being incubated with ab197956 overnight at 4°C. Antibody binding was visualised using ECL development solution ab133406.

References

ab197956 has not yet been referenced specifically in any publications.

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