Anti-Pyruvate Dehydrogenase E2 抗体 (ab103948)

製品の概要

  • 製品名Anti-Pyruvate Dehydrogenase E2 antibody
    Pyruvate Dehydrogenase E2 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to Pyruvate Dehydrogenase E2
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
  • 免疫原

    Synthetic peptide corresponding to a region within C terminal amino acids 587-617 of Human Pyruvate Dehydrogenase E2, conjugated to KLH.

  • ポジティブ・コントロール
    • HepG2 cell lysate

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab103948 in the following tested applications.

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

アプリケーション Abreviews 特記事項
WB 1/100 - 1/500. Predicted molecular weight: 69 kDa.

ターゲット情報

  • 機能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).
  • 関連疾患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.
  • 配列類似性Belongs to the 2-oxoacid dehydrogenase family.
    Contains 2 lipoyl-binding domains.
  • 細胞内局在Mitochondrion matrix.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 70 kDa mitochondrial autoantigen of primary biliary cirrhosis antibody
    • Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex antibody
    • Dihydrolipoamide S Acetyltransferase antibody
    • Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex antibody
    • dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial antibody
    • DLAT antibody
    • DLTA antibody
    • E2 antibody
    • E2 component of pyruvate dehydrogenase complex antibody
    • M2 antigen complex 70 kDa subunit antibody
    • mitochondrial antibody
    • ODP2_HUMAN antibody
    • PBC antibody
    • PDC E2 antibody
    • PDC-E2 antibody
    • PDCE2 antibody
    • Pyruvate dehydrogenase complex component E2 antibody
    • Pyruvate dehydrogenase complex E2 subunit antibody
    see all

Anti-Pyruvate Dehydrogenase E2 antibody 画像

  • Anti-Pyruvate Dehydrogenase E2 antibody (ab103948) at 1/100 dilution + HepG2 cell lysate at 35 µg

    Predicted band size : 69 kDa

Anti-Pyruvate Dehydrogenase E2 antibody (ab103948) 使用論文

ab103948 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab103948.
Please use the links above to contact us or submit feedback about this product.

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