Recombinant人Pyruvate Dehydrogenase E2 protein (ab125824)

概述

描述

  • 性质
    Recombinant
  • 来源
    Insect cells
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 分子量
      61 kDa
    • 氨基酸
      87 to 647
    • 额外的序列信息
      DLAT 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).

技术指标

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.

  • 应用

    SDS-PAGE

    Western blot

    ELISA

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

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • 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
  • 功能
    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).
  • 疾病相关
    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.
  • 序列相似性
    Belongs to the 2-oxoacid dehydrogenase family.
    Contains 2 lipoyl-binding domains.
  • 细胞定位
    Mitochondrion matrix.
  • Information by UniProt

文献

ab125824 has not yet been referenced specifically in any publications.

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