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MitoSciences (MS964)

PDH E1 alpha protein (PDHA1) ELISA Kit (ab115342)

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Overview

  • Product namePDH E1 alpha protein (PDHA1) ELISA KitSee all Pyruvate Dehydrogenase E1-alpha subunit kits ...
  • Precision
    Intra-assay
    Sample n Mean SD CV%
    1 8 = 2.3%
    Inter-assay
    Sample n Mean SD CV%
    1 4 = 8.2%
  • Tests
    1 x 96 test
  • Sample type
    Cell culture extracts, Tissue Extracts
  • Assay typeQuantitative
  • Sensitivity
    = 15 ng/ml
  • Species reactivity
    Reacts with: Mouse, Rat, Cow, Human
  • Product overview

    ab115342 is an in vitro enzyme-linked immunosorbent assay for the quantitative measurement of PDHA1 protein in cell and tissue lysates. The assay employs an antibody specific for PDHA1 protein coated on a 96-well plate. Standards and samples are pipetted into the wells and PDHA1 protein present in the sample is bound to the wells by the immobilized antibody. The wells are washed and a biotin labeled anti-PDHA1 protein detector antibody is added. After washing away unbound detector antibody, HRP-conjugated streptavidin specific for the biotin labeled detector antibody is pipetted into the wells. The wells are again washed, an HRP substrate solution (TMB) is added to the wells and color develops in proportion to the amount of PDHA1 protein bound. The developing blue color is measured at 600 nm. Optionally the reaction can be stopped by adding hydrochloric acid which changes the color from blue to yellow and the intensity can be measured at 450 nm.

  • Notes

    Store all components at 4°C. This kit is stable for at least 6 months

    from receipt. After reconstitution the standard should be stored at -

    80°C. Unused microplate strips should be returned to the pouch

    containing the desiccant and resealed.

  • Tested applicationsELISA more details

Properties

  • 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).
  • Tissue specificityUbiquitous.
  • Involvement in diseaseDefects in PDHA1 are a cause of pyruvate dehydrogenase E1-alpha deficiency (PDHAD) [MIM:312170]. An enzymatic defect causing primary lactic acidosis in children. It is associated with a broad clinical spectrum ranging from fatal lactic acidosis in the newborn to chronic neurologic dysfunction with structural abnormalities in the central nervous system without systemic acidosis.
    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.
  • Post-translational
    modifications    Phosphorylation at Ser-293 by PDK family kinases blocks the access to active site, and inactivates the enzyme.
  • Cellular localizationMitochondrion matrix.

    • Target information above from: UniProt accession P08559 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt
    • Alternative names
        mitochondrialMS964ODPA_HUMAN
        PDHA1PDHE1-A type IPyruvate dehydrogenase E1 component subunit alphasomatic form
      see all
  • Database links

      • Applications

      Our Abpromise guarantee covers the use of ab115342 in the following tested applications.

      The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

      Application Notes
      ELISA ELISA

      PDH E1 alpha protein (PDHA1) ELISA Kit images

      • ELISA - PDH E1 alpha protein (PDHA1) ELISA Kit (ab115342)
        ELISA - PDH E1 alpha protein (PDHA1) ELISA Kit (ab115342)
        Example of standard curve constructed using ab115342.
      • ELISA - PDH E1 alpha protein (PDHA1) ELISA Kit (ab115342)
        ELISA - PDH E1 alpha protein (PDHA1) ELISA Kit (ab115342)
        Example HeLa dilution series and working range produced with ab115342.
      • ELISA - PDH E1 alpha protein (PDHA1) ELISA Kit (ab115342)
        ELISA - PDH E1 alpha protein (PDHA1) ELISA Kit (ab115342)
        An experiment to determine the levels of PDHA1 in various fibroblast cell lines. A fibroblast cell line (P) harboring a mutation in the X-chromosome PDHA1 gene results in significant reduction in E1a protein expression. This is compared to control adult fibroblast cells (control) and mouse fibroblast cells (NIH3T3 and MEF, mouse embryonic fibroblasts).

      Protocols

      References for PDH E1 alpha protein (PDHA1) ELISA Kit (ab115342)

      ab115342 has not yet been referenced specifically in any publications.

      Publishing research using ab115342 ? Please let us know so that we can cite the reference in this datasheet

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