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Anti-PDHA1/FITC抗体

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  • ¥2980
  • LMAI Bio
  • LM-4034R-FITC
  • 中国/美国/欧洲
  • 2025年07月15日
  • ICC=1:50-200 IF=1:50-200
  • Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,
  • Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,
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    • 详细信息
    • 文献和实验
    • 技术资料
    • 供应商

      上海联迈生物工程有限公司

    • 库存

      大量

    • 靶点

      详见说明书

    • 级别

      1

    • 目录编号

      LM-4034R-FITC

    • 克隆性

      多克隆

    • 抗原来源

      Rabbit

    • 保质期

      1年

    • 抗体英文名

      Anti-PDHA1/FITC

    • 抗体名

      Anti-PDHA1/FITC

    • 标记物

      FITC标记

    • 宿主

      Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,

    • 适应物种

      Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,

    • 免疫原

      详见说明书

    • 亚型

      IGg

    • 形态

      粉末、液体、冻干粉

    • 应用范围

      ICC=1:50-200 IF=1:50-200

    • 浓度

      1mg/ml

    • 保存条件

      -20 °C

    • 规格

      100ul

    Anti-PDHA1/FITC抗体
    英文名称 Anti-PDHA1/FITC
    中文名称 Anti-PDHA1/FITC抗体
    别    名 mitochondrial; somatic form; ODPA_HUMAN; PDH; PDHA1; PDHCE1A; PDHE1 A type I; PDHE1-A type I; PHE1A; Pyruvate Dehydrogenase (lipoamide) alpha 1; Pyruvate Dehydrogenase E1 alpha; Pyruvate dehydrogenase E1 component subunit alpha; Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial.  
    规格价格 100ul/2980元 购买        大包装/询价
    说 明 书 100ul  
    研究领域 肿瘤  细胞生物  免疫学  激酶和磷酸酶  线粒体  
    抗体来源 Rabbit
    克隆类型 Polyclonal
    交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, 
    产品应用 ICC=1:50-200 IF=1:50-200  
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 43kDa
    性    状 Lyophilized or Liquid
    浓    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human PDHA1
    亚    型 IgG
    纯化方法 affinity purified by Protein A
    储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
    产品介绍 background:
    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.

    Function:
    The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.

    Subunit:
    Tetramer of 2 alpha and 2 beta subunits.

    Subcellular Location:
    Mitochondrion matrix.

    Tissue Specificity:
    Testis. Expressed in postmeiotic spermatogenic cells.

    Post-translational modifications:
    Phosphorylation at Ser-232, Ser-293 and Ser-300 by PDK family kinases inactivates the enzyme; for this phosphorylation at a single site is sufficient. Dephosphorylation at all three sites, i.e. at Ser-232, Ser-293 and Ser-300, is required for reactivation. 

    DISEASE:
    Pyruvate dehydrogenase E1-alpha deficiency (PDHAD) [MIM:312170]: An enzymatic defect causing primary lactic acidosis in children. It is associated with a broad clinical spectrum ranging from fatal lactic acidosis in the newborn to chronic neurologic dysfunction with structural abnormalities in the central nervous system without systemic acidosis. Note=The disease is caused by mutations affecting the gene represented in this entry. 
    X-linked Leigh syndrome (X-LS) [MIM:308930]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry. 

    Database links:

    Entrez Gene: 407109 Cow

    Entrez Gene: 5160 Human

    Entrez Gene: 18597 Mouse

    Entrez Gene: 29554 Rat

    Omim: 300502 Human

    SwissProt: A7MB35 Cow

    SwissProt: P08559 Human

    SwissProt: P35486 Mouse

    SwissProt: P26284 Rat

    Unigene: 530331 Human

    Unigene: 34775 Mouse

    Unigene: 3655 Rat



    Important Note:
    This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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