Recombinant人ACADVL protein (ab98234)

概述

  • 产品名称
    Recombinant人ACADVL protein
  • 蛋白长度
    Full length protein

描述

  • 性质
    Recombinant
  • 来源
    Escherichia coli
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      MGSSHHHHHHSSGLVPRGSHMAGGAAQLALDKSDSHPSDALTRKKPAKAE SKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDP AKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGM HDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEP SSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTD PATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPS ENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQF GEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISK IFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILR LFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGL SLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLA DGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMA ALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF
    • 分子量
      69 kDa including tags
    • 氨基酸
      41 to 655
    • 标签
      His tag N-Terminus

技术指标

Our Abpromise guarantee covers the use of ab98234 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

  • 纯度
    > 90 % SDS-PAGE.
    ab98234 was 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.

    Preservative: None
    Constituents: 10% Glycerol, 0.1M Sodium chloride, 20mM Tris HCl, 1mM EDTA, 1mM DTT, pH 8.0

常规信息

  • 别名
    • ACAD 6
    • ACAD6
    • ACADV_HUMAN
    • Acadvl
    • Acyl CoA dehydrogenase very long chain
    • Acyl Coenzyme A dehydrogenase very long chain
    • LCACD
    • mitochondrial
    • Very long chain specific acyl CoA dehydrogenase
    • Very long chain specific acyl CoA dehydrogenase mitochondrial
    • Very long-chain specific acyl-CoA dehydrogenase
    • VLCAD
    see all
  • 功能
    Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accomodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.
  • 通路
    Lipid metabolism; mitochondrial fatty acid beta-oxidation.
  • 疾病相关
    Defects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD) [MIM:201475]. ACADVLD is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and 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 inner membrane.
  • Information by UniProt

Recombinant Human ACADVL protein 图像

  • 15% SDS-PAGE analysis of 3µg ab98234

Recombinant Human ACADVL protein (ab98234)参考文献

ab98234 has not yet been referenced specifically in any publications.

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