Recombinant人Pyruvate Dehydrogenase E1-alpha subunit protein (ab91940)

概述

描述

  • 性质Recombinant
  • 来源Escherichia coli
  • 氨基酸序列
    • 种属Human
    • 序列MRKMLAAVSRVLSGASQKPASRVLVASRNFANDATFEIKKCDLHRLEEGP PVT TVLTREDGLKYYRMMQTVRRMELKADQLYKQKIIRGFCHLCDGQE ACCVGLEA GINPTDHLITAYRAHGFTFTRGLSVREILAELTGRKGGCA KGKGGSMHMYAKN FYGGNGIVGAQVPLGAGIALACKYNGKDEVCLTLY GDGAANQGQIFEAYNMAA LWKLPCIFICENNRYGMGTSVERAAASTDY YKRGDFIPGLRVDGMDILCVREA TRFAAAYCRSGKGPILMELQTYRYH GHSMSDPGVSYRTREEIQEVRSKSDPIM LLKDRMVNSNLASVEELKEI DVEVRKEIEDAAQFATADPEPPLEELGYHIYSS DPPFEVRGANQWIKF KSVS

技术指标

Our Abpromise guarantee covers the use of ab91940 in the following tested applications.

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

  • 应用

    Western blot

    Mass Spectrometry

    SDS-PAGE

  • 形式Lyophilised
  • 补充说明Protein Identity confirmed by Mass Spectrometry (MS/MS) (acquired on initial reference batch)
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    Preservative: None
    Constituents: 0.5% Trehalose, 6M Urea, 100mM Sodium phosphate, 10mM Sodium chloride, pH 4.5

  • 复溶Reconstitute with 160 µl aqua dest.

常规信息

  • 别名
    • ODPA_HUMAN
    • PDH
    • PDHA
    • PDHA1
    • PDHCE1A
    • PDHE1 A type I
    • PDHE1-A type I
    • PHE1A
    • Pyruvate Dehydrogenase (lipoamide) alpha 1
    • Pyruvate dehydrogenase complex, E1 alpha polypeptide 1
    • Pyruvate Dehydrogenase E1 alpha
    • Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
    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).
  • 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

Recombinant Human Pyruvate Dehydrogenase E1-alpha subunit protein 图像

  • The image shows an electrophoretic assay performed using an Agilent 5100 ALP. In some images coloured control bands can be seen at 15 kDa (green) and/or 240 kDa (purple). The protein-specific band is blue.
  • 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

Recombinant Human Pyruvate Dehydrogenase E1-alpha subunit protein (ab91940)参考文献

ab91940 has not yet been referenced specifically in any publications.

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To our knowledge, the protein has only been tested by SDS-PAGE. It has not been tested for enzymatic activity, however, we expect no activity since it was purified under denaturing conditions.

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