製品の概要

  • 製品名
    Recombinant Human ACADVL protein
  • タンパク質長
    Full length protein

製品の詳細

  • 由来
    Recombinant
  • 由来
    Escherichia coli
  • アミノ酸配列
    • アクセッション番号
    • 生物種
      Human
    • 配列
      MGSSHHHHHHSSGLVPRGSHMAGGAAQLALDKSDSHPSDALTRKKPAKAE SKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDP AKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGM HDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEP SSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTD PATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPS ENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQF GEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISK IFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILR LFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGL SLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLA DGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMA ALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF
    • 分子量
      69 kDa including tags
    • 領域
      41 to 655
    • タグ
      His tag N-Terminus

特性

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.

  • アプリケーション

    SDS-PAGE

  • 精製度
    > 90 % SDS-PAGE.
    ab98234 was purified using conventional chromatography techniques.
  • 製品の状態
    Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    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

関連情報

  • 別名
    • 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
  • 機能
    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.
  • パスウェイ
    Lipid metabolism; mitochondrial fatty acid beta-oxidation.
  • 関連疾患
    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.
  • 配列類似性
    Belongs to the acyl-CoA dehydrogenase family.
  • 細胞内局在
    Mitochondrion inner membrane.
  • Information by UniProt

画像

  • 15% SDS-PAGE analysis of 3µg ab98234

参考文献

ab98234 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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