Anti-Pyruvate Dehydrogenase E1-alpha subunit 抗体 (ab97352)

製品の概要

  • 製品名Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody
    Pyruvate Dehydrogenase E1-alpha subunit 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to Pyruvate Dehydrogenase E1-alpha subunit
  • アプリケーション適用あり: WB, ICC/IFmore details
  • 種交差性
    交差種: Rat, Human
    交差が予測される動物種: Mouse, Cow, Zebrafish
  • 免疫原

    Recombinant fragment, corresponding to a region within amino acids 39-295 of Human Pyruvate Dehydrogenase E1-alpha subunit (NP_000275).

  • ポジティブ・コントロール
    • WB: 293T, A431, H1299, HeLa S3, HepG2, MOLT4 and Raji ICC/IF: HeLa cells

法規制情報

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab97352 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 1/500 - 1/3000. Predicted molecular weight: 43 kDa.
ICC/IF 1/100 - 1/200.

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).
  • 組織特異性Ubiquitous.
  • 関連疾患Defects in PDHA1 are a cause of pyruvate decarboxylase E1 component deficiency (PDHE1 deficiency) [MIM:312170]. PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (X-LS).
    Defects in PDHA1 are the cause of X-linked Leigh syndrome (X-LS) [MIM:308930]. X-LS is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord. The lesions are areas of demyelination, gliosis, necrosis, spongiosis, or capillary proliferation. Clinical symptoms depend on which areas of the central nervous system are involved. The most common underlying cause is a defect in oxidative phosphorylation. LS may be a feature of a deficiency of any of the mitochondrial respiratory chain complexes.
  • 細胞内局在Mitochondrion matrix.
  • Information by UniProt
  • 参照データベース
  • 別名
    • ODPA_HUMAN antibody
    • PDH antibody
    • PDHA antibody
    • PDHA1 antibody
    • PDHCE1A antibody
    • PDHE1 A type I antibody
    • PDHE1-A type I antibody
    • PHE1A antibody
    • Pyruvate Dehydrogenase (lipoamide) alpha 1 antibody
    • Pyruvate dehydrogenase complex, E1 alpha polypeptide 1 antibody
    • Pyruvate Dehydrogenase E1 alpha antibody
    • Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial antibody
    see all

Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody 画像

  • All lanes : Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody (ab97352) at 1/1000 dilution

    Lane 1 : HepG2 whole cell lysate
    Lane 2 : MOLT4 whole cell lysate

    Lysates/proteins at 30 µg per lane.


    Predicted band size : 43 kDa
  • ab97352, at a 1/200 dilution, staining Pyruvate Dehydrogenase E1-alpha subunit in paraformaldehyde fixed HeLa cells by Immunofluorescence. Lower imagewas merged with DNA probe.

Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody (ab97352) 使用論文

This product has been referenced in:
  • Macdonald MJ  et al. Differences between Human and Rodent Pancreatic Islets: LOW PYRUVATE CARBOXYLASE, ATP CITRATE LYASE, AND PYRUVATE CARBOXYLATION AND HIGH GLUCOSE-STIMULATED ACETOACETATE IN HUMAN PANCREATIC ISLETS. J Biol Chem 286:18383-96 (2011). WB ; Human, Rat . Read more (PubMed: 21454710) »

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