Description

  • Product name

    Recombinant Mouse Pyruvate Dehydrogenase E2 protein
    See all Pyruvate Dehydrogenase E2 proteins and peptides
  • Purity

    > 90 % SDS-PAGE.

  • Expression system

    Mammalian
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Mouse
    • Sequence

      SLPPHQKVPLPSLSPTMQAGTIARWEKKEGEKISEGDLIAEVETDKATVG FESLEECYMAKILVPEGTRDVPVGSIICITVEKPQDIEAFKNYTLDLAAA AAPQAAPAAAPAPAAAPAAPSASAPGSSYPTHMQIVLPALSPTMTMGTVQ RWEKKVGEKLSEGDLLAEIETDKATIGFEVQEEGYLAKILVPEGTRDVPL GAPLCIIVEKQEDIAAFADYRPTEVTSLKPQAAPPAPPPVAAVPPTPQPV APTPSAAPAGPKGRVFVSPLAKKLAAEKGIDLTQVKGTGPEGRIIKKDID SFVPSKAAPAAAAAMAPPGPRVAPAPAGVFTDIPISNIRRVIAQRLMQSK QTIPHYYLSVDVNMGEVLLVRKELNKMLEGKGKISVNDFIIKASALACLK VPEANSSWMDTVIRQNHVVDVSVAVSTPAGLITPIVFNAHIKGLETIASD VVSLASKAREGKLQPHEFQGGTFTISNLGMFGIKNFSAIINPPQACILAI GASEDKLIPADNEKGFDVASVMSVTLSCDHRVVDGAVGAQWLAEFKKYLE KPITMLL
    • Predicted molecular weight

      59 kDa
    • Amino acids

      86 to 642
    • Additional sequence information

      This product is the mature full length protein from aa 86 to 642. The transit peptide is not included.

Specifications

Our Abpromise guarantee covers the use of ab225979 in the following tested applications.

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

  • Applications

    SDS-PAGE

  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    Constituents: 50% Glycerol, Tris buffer

General Info

  • Alternative names

    • 70 kDa mitochondrial autoantigen of primary biliary cirrhosis
    • Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex
    • Dihydrolipoamide S Acetyltransferase
    • Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex
    • dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial
    • DLAT
    • DLTA
    • E2
    • E2 component of pyruvate dehydrogenase complex
    • M2 antigen complex 70 kDa subunit
    • mitochondrial
    • ODP2_HUMAN
    • PBC
    • PDC E2
    • PDC-E2
    • PDCE2
    • Pyruvate dehydrogenase complex component E2
    • Pyruvate dehydrogenase complex E2 subunit
    see all
  • 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

References

ab225979 has not yet been referenced specifically in any publications.

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