Recombinant人Pyruvate Dehydrogenase E1-alpha subunit protein (ab125602)

概述

描述

  • 性质Recombinant
  • 来源Escherichia coli
  • 氨基酸序列
    • AccessionP08559
    • 种属Human
    • 分子量47 kDa
    • 氨基酸30 to 390
    • 标签His tag N-Terminus

技术指标

Our Abpromise guarantee covers the use of ab125602 in the following tested applications.

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

  • 应用

    Functional Studies

    Western blot

    SDS-PAGE

  • 纯度> 85 % Densitometry.

  • 形式Liquid
  • 补充说明

    ab125602 (Human Pyruvate Dehydrogenase E1-alpha subunit full length protein) can be utilized as a substrate for the following active protein Kinases:

     

    ab125560 (Active human PDK4 full length protein)

    ab125580 (Active human Mitochondrial Pyruvate dehydrogenase kinase 1 full length protein)
    ab125592 (Active human PDK2 full length protein)
    ab125606 (Active human PDK3 full length protein)

     

  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 7.00
    Preservative: 1.02% Imidazole
    Constituents: 0.002% PMSF, 0.81% Sodium phosphate, 0.004% DTT, 25% Glycerol, 1.75% Sodium chloride

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

Recombinant Human Pyruvate Dehydrogenase E1-alpha subunit protein (ab125602)参考文献

ab125602 has not yet been referenced specifically in any publications.

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