Recombinant Human Pyruvate Dehydrogenase E2 protein (ab125824)

Overview

Description

  • NatureRecombinant
  • SourceInsect cells
  • Amino Acid Sequence
    • AccessionP10515
    • SpeciesHuman
    • Molecular weight61 kDa
    • Amino acids87 to 647
    • Additional sequence informationDLAT is a full-length cDNA coding for the mature form of the human PDC-E2 protein having a molecular mass of 60,630 Dalton (pH 5.8).

Specifications

Our Abpromise guarantee covers the use of ab125824 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

    Western blot

    ELISA

  • Purity> 75 % SDS-PAGE.
    Purity is greater than 75.0% as determined by SDS-PAGE.
  • FormLiquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.

    pH: 8.00
    Constituents: 0.48% HEPES, 20% Glycerol, 1.17% Sodium chloride

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
  • 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).
  • Involvement in diseaseNote=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 similaritiesBelongs to the 2-oxoacid dehydrogenase family.
    Contains 2 lipoyl-binding domains.
  • Cellular localizationMitochondrion matrix.
  • Information by UniProt

References for Recombinant Human Pyruvate Dehydrogenase E2 protein (ab125824)

ab125824 has not yet been referenced specifically in any publications.

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