Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody [8D10E6] (Alexa Fluor® 488) (ab197961)

Overview

  • Product name

    Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody [8D10E6] (Alexa Fluor® 488)
    See all Pyruvate Dehydrogenase E1-alpha subunit primary antibodies
  • Description

    Mouse monoclonal [8D10E6] to Pyruvate Dehydrogenase E1-alpha subunit (Alexa Fluor® 488)
  • Host species

    Rabbit
  • Conjugation

    Alexa Fluor® 488. Ex: 495nm, Em: 519nm
  • Tested applications

    Suitable for: ICC/IFmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Cow, Human, Caenorhabditis elegans, Drosophila melanogaster
  • Immunogen

    Full length native protein (purified) corresponding to Human Pyruvate Dehydrogenase E1-alpha subunit.

  • Positive control

    • ICC/IF: HeLa cells.
  • General notes

    Alexa Fluor® is a registered trademark of Molecular Probes, Inc, a Thermo Fisher Scientific Company. The Alexa Fluor® dye included in this product is provided under an intellectual property license from Life Technologies Corporation. As this product contains the Alexa Fluor® dye, the purchase of this product conveys to the buyer the non-transferable right to use the purchased product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). As this product contains the Alexa Fluor® dye the sale of this product is expressly conditioned on the buyer not using the product or its components, or any materials made using the product or its components, in any activity to generate revenue, which may include, but is not limited to use of the product or its components: in manufacturing; (ii) to provide a service, information, or data in return for payment (iii) for therapeutic, diagnostic or prophylactic purposes; or (iv) for resale, regardless of whether they are sold for use in research. For information on purchasing a license to this product for purposes other than research, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@thermofisher.com.

Properties

Applications

Our Abpromise guarantee covers the use of ab197961 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
ICC/IF 1/100.

This product gave a positive signal in HeLa cells fixed with 4% formaldehyde (10 min).

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

  • ab197961 staining Pyruvate Dehydrogenase E1-alpha subunit in HeLa cells. The cells were fixed with 4% formaldehyde (10 min), permeabilized with 0.1% Triton X-100 for 5 minutes and then blocked with 1% BSA/10% normal goat serum/0.3M glycine in 0.1%PBS-Tween for 1h. The cells were then incubated overnight at +4°C with ab197961 at a 1/100 dilution (shown in green) and ab195889, Mouse monoclonal to alpha Tubulin (Alexa Fluor® 594), at a 1/250 dilution (shown in red). Nuclear DNA was labelled with DAPI (shown in blue).

    Image was taken with a confocal microscope (Leica-Microsystems, TCS SP8).

References

ab197961 has not yet been referenced specifically in any publications.

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