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Pyruvate Dehydrogenase E1 α (11V10) Rabbit Monoclonal Antibody  Cite:0    分享

货号： IM68849
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  • 种属：H，M，R
  • 用途：WB，ELISA，IP，IHC-P，IF-P，IF-F，IF-ICC
规格:
50μl 100μl
单位:
支
单价:￥1300.00
产品简介
储存与保存
注意事项
产品简介
  • 产品货号
    IM68849
  • 别名
    PDHA1;PHE1A;Pyruvate dehydrogenase E1 component subunit alpha; somatic form, mitochondrial;PDHE1-A type I
  • 产品名称
    Pyruvate Dehydrogenase E1 α (11V10) Rabbit Monoclonal Antibody
  • 类别
    抗体产品
  • 蛋白名称
    PDHA1 ODPA
  • Clonality
    Monoclonal
  • 推荐应用
    WB,IHC-P,IF-P,IF-F,IF-ICC,IP,ELISA
  • 反应种属
    Human,Mouse,Rat
  • 存储缓冲液
    PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
  • Human Gene ID
    5160
  • Human Swissprot No.
    P08559
  • Mouse Gene ID
    18597
  • Mouse Swissprot No.
    P35486
  • Rat Swissprot No.
    P26284
  • 稀释度
    IHC-P 1:200-1:1000,WB 1:1000-1:5000,IF-P/IF-F/IF-ICC 1:200-1:1000,ELISA 1:5000-1:20000,IP 1:50-1:200
  • 参考分子量
    43kDa
  • 预测分子量
    43kDa
  • 序列
    Pyruvate dehydrogenase E1 component subunit alpha somatic form mitochondrial
  • 宿主
    Rabbit
  • 同种型
    Monoclonal, rabbit, IgG, Kappa
  • 背景介绍
    The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 alpha 1 subunit containing the E1 active site, and plays a key role in the function of the PDH complex. Mutations in this gene are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Mar 2010].
  • 信号通路
    Glycolysis / Gluconeogenesis;Citrate cycle (TCA cycle);Valine; leucine and isoleucine biosynthesis;Pyruvate metabolism;Butanoate metabolism.
  • 功能
    catalytic activity:Pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO(2).,cofactor:Thiamine pyrophosphate.,disease: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 (LS) (Leigh encephalomyelopathy).,disease:Defects in PDHA1 are the cause of X-linked Leigh syndrome (LS) [MIM:308930]. 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.,enzyme regulation:E1 activity is regulated by phosphorylation (inactivation) and dephosphorylation (activation) of the alpha subunit.,function: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).,subunit:Tetramer of 2 alpha and 2 beta subunits.,tissue specificity:Ubiquitous.
  • 纯化
    Protein A
储存与保存

1.保存：-20℃

2.有效期：1年

注意事项

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2.本产品仅供科研使用。请勿用于医药、临床诊断或治疗，食品及化妆品等用途。请勿存放于普通住宅区。 

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