Recombinant人ACADL protein (ab113579)

概述

描述

  • 性质
    Recombinant
  • 来源
    Escherichia coli
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      MGSSHHHHHHSSGLVPRGSHMGGEERLETPSAKKLTDIGIRRIFSPEHDI FRKSVRKFFQEEVIPHHSEWEKAGEVSREVWEKAGKQGLLGVNIAEHLGG IGGDLYSAAIVWEEQAYSNCSGPGFSIHSGIVMSYITNHGSEEQIKHFIP QMTAGKCIGAIAMTEPGAGSDLQGIKTNAKKDGSDWILNGSKVFISNGSL SDVVIVVAVTNHEAPSPAHGISLFLVENGMKGFIKGRKLHKMGLKAQDTA ELFFEDIRLPASALLGEENKGFYYIMKELPQERLLIADVAISASEFMFEE TRNYVKQRKAFGKTVAHLQTVQHKLAELKTHICVTRAFVDNCLQLHEAKR LDSATACMAKYWASELQNSVAYDCVQLHGGWGYMWEYPIAKAYVDARVQP IYGGTNEIMKELIAREIVFDK
    • 分子量
      47 kDa including tags
    • 氨基酸
      31 to 430
    • 标签
      His tag N-Terminus

技术指标

Our Abpromise guarantee covers the use of ab113579 in the following tested applications.

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

  • 应用

    SDS-PAGE

    Mass Spectrometry

  • 质谱法
    MALDI-TOF
  • 纯度
    > 85 % SDS-PAGE.
    > 85 % by SDS - PAGE. ab113579 is purified using conventional chromatography techniques.
  • 形式
    Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 8.00
    Constituents: 0.32% Tris HCl, 10% Glycerol, 0.02% DTT, 0.88% Sodium chloride

常规信息

  • 别名
    • ACAD4
    • ACADL
    • ACADL_HUMAN
    • Acyl Coenzyme A dehydrogenase long chain
    • Acyl-CoA dehydrogenase long chain
    • FLJ94052
    • LCAD
    • Long chain acyl CoA dehydrogenase
    • Long-chain specific acyl-CoA dehydrogenase, mitochondrial
    see all
  • 通路
    Lipid metabolism; mitochondrial fatty acid beta-oxidation.
  • 疾病相关
    Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]. An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting.
  • 序列相似性
    Belongs to the acyl-CoA dehydrogenase family.
  • 细胞定位
    Mitochondrion matrix.
  • Information by UniProt

图片

  • 15% SDS-PAGE showing ab113579 (3µg).

文献

ab113579 has not yet been referenced specifically in any publications.

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