Recombinant Human Pyruvate Dehydrogenase E2 protein (ab158286)

Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MWRVCARRAQNVAPWAGLEARWTALQEVPGTPRVTSRSGPAPARRNSVTT GYGGVRALCGWTPSSGATPRNRLLLQLLGSPGRRYYSLPPHQKVPLPSLS PTMQAGTIARWEKKEGDKINEGDLIAEVETDKATVGFESLEECYMAKILV AEGTRDVPIGAIICITVGKPEDIEAFKNYTLDSSAAPTPQAAPAPTPAAT ASPPTPSAQAPGSSYPPHMQVLLPALSPTMTMGTVQRWEKKVGEKLSEGD LLAEIETDKATIGFEVQEEGYLAKILVPEGTRDVPLGTPLCIIVEKEADI SAFADYRPTEVTDLKPQVPPPTPPPVAAVPPTPQPLAPTPSAPCPATPAG PKGRVFVSPLAKKLAVEKGIDLTQVKGTGPDGRITKKDIDSFVPSKVAPA PAAVVPPTGPGMAPVPTGVFTDIPISNIRRVIAQRLMQSKQTIPHYYLSI DVNMGEVLLVRKELNKILEGRSKISVNDFIIKASALACLKVPEANSSWMD TVIRQNHVVDVSVAVSTPAGLITPIVFNAHIKGVETIANDVVSLATKARE GKLQPHEFQGGTFTISNLGMFGIKNFSAIINPPQACILAIGASEDKLVPA DNEKGFDVASMMSVTLSCDHRVVDGAVGAQWLAEFRKYLEKPITMLL
    • Amino acids
      1 to 647
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab158286 in the following tested applications.

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

  • Applications

    Western blot

    ELISA

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

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

Images

  • ab158286 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab158286 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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