重组人Pyruvate Dehydrogenase E1 beta subunit蛋白(ab152601)

概述

  • 产品名称
    重组人Pyruvate Dehydrogenase E1 beta subunit蛋白
  • 蛋白长度
    Protein fragment

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • 种属
      Human
    • 序列
      LEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQF GVGAEICARIMEGPAFNFLDAPAVRVTGADVPMPYAKILEDNSIPQVKDI IFAIKKTLNI
    • 氨基酸
      250 to 359
    • 标签
      proprietary tag N-Terminus

技术指标

Our Abpromise guarantee covers the use of ab152601 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

    ELISA

  • 形式
    Liquid
  • 补充说明
    Protein concentration is above or equal to 0.05 mg/ml.
  • 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: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • DKFZp564K0164
    • mitochondrial
    • ODPB_HUMAN
    • pdhB
    • PDHBD
    • PDHE1 B
    • PDHE1-B
    • PHE1B
    • Pyruvate dehydrogenase (lipoamide) beta
    • Pyruvate dehydrogenase E1 beta polypeptide
    • Pyruvate dehydrogenase E1 component subunit beta
    • Pyruvate dehydrogenase E1 component subunit beta 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).
  • 疾病相关
    Defects in PDHB are a cause of pyruvate dehydrogenase 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).
  • 细胞定位
    Mitochondrion matrix.
  • Information by UniProt

图片

  • ab152601 on a 12.5% SDS-PAGE stained with Coomassie Blue.

文献

ab152601 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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