Anti-Pyruvate Dehydrogenase E1-alpha subunit抗体(ab92695)

概述

  • 产品名称Anti-Pyruvate Dehydrogenase E1-alpha subunit抗体
    参阅全部 Pyruvate Dehydrogenase E1-alpha subunit 一抗
  • 描述
    兔多克隆抗体to Pyruvate Dehydrogenase E1-alpha subunit
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Mouse, Rat, Human
    预测可用于: Pig, Chimpanzee
  • 免疫原

    Synthetic peptide conjugated to KLH derived from within residues 200 - 300 of Human Pyruvate Dehydrogenase E1-alpha subunit.

  • 阳性对照
    • This antibody gave a positive signal in Mouse Skeletal Muscle and Rat Heart Tissue Lysates as well as in the following whole cell lysates: HepG2; HeLa; HEK293; MOLT4; NIH3T3; PC12.

性能

应用

Our Abpromise guarantee covers the use of ab92695 in the following tested applications.

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

应用 Ab评论 说明
WB Use a concentration of 1 µg/ml. Detects a band of approximately 43 kDa (predicted molecular weight: 43 kDa).

靶标

  • 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).
  • 组织特异性Ubiquitous.
  • 疾病相关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.
  • 细胞定位Mitochondrion matrix.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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 图像

  • All lanes : Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody (ab92695) at 1 µg/ml

    Lane 1 : HepG2 (Human hepatocellular liver carcinoma cell line) Whole Cell Lysate
    Lane 2 : HeLa (Human epithelial carcinoma cell line) Whole Cell Lysate
    Lane 3 : HEK293 (Human embryonic kidney cell line) Whole Cell Lysate
    Lane 4 : MOLT4 (Human acute lymphoblastic leukemia cell line) Whole Cell Lysate

    Lysates/proteins at 10 µg per lane.

    Secondary
    Goat polyclonal Secondary Antibody to Rabbit IgG - H&L (HRP), pre-adsorbed at 1/5000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 43 kDa
    Observed band size : 46 kDa (why is the actual band size different from the predicted?)
    Additional bands at : 100 kDa. We are unsure as to the identity of these extra bands.

    Exposure time : 12 minutes
  • Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody (ab92695) at 1 µg/ml + Recombinant Human Pyruvate Dehydrogenase E1-alpha subunit protein (ab91940) at 0.01 µg

    Secondary
    Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Exposure time : 1 minute
  • All lanes : Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody (ab92695) at 1 µg/ml

    Lane 1 : NIH 3T3 (Mouse embryonic fibroblast cell line) Whole Cell Lysate
    Lane 2 : Skeletal Muscle (Mouse) Tissue Lysate
    Lane 3 : PC12 (Rat adrenal pheochromocytoma cell line) Whole Cell Lysate
    Lane 4 : Heart (Rat) Tissue Lysate

    Lysates/proteins at 10 µg per lane.

    Secondary
    Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 43 kDa
    Observed band size : 43 kDa
    Additional bands at : 100 kDa. We are unsure as to the identity of these extra bands.

    Exposure time : 1 minute

Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody (ab92695)参考文献

ab92695 has not yet been referenced specifically in any publications.

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