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Recombinant Human ACADVL/VLCAD protein (ab98234)

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SDS-PAGE - Recombinant Human ACADVL/VLCAD protein (ab98234)

    Key features and details

    • Expression system: Escherichia coli
    • Purity: > 90% SDS-PAGE
    • Tags: His tag N-Terminus
    • Suitable for: SDS-PAGE

    製品の詳細

    • 製品名

      Recombinant Human ACADVL/VLCAD protein

    • 精製度

      > 90 % SDS-PAGE.
      ab98234 was purified using conventional chromatography techniques.

    • 発現系

      Escherichia coli

    • アクセッション番号

      P49748

    • タンパク質長

      Full length protein

    • Animal free

      No

    • 由来

      Recombinant

      • 生物種

        Human

      • 配列

        MGSSHHHHHHSSGLVPRGSHMAGGAAQLALDKSDSHPSDALTRKKPAKAE SKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDP AKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGM HDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEP SSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTD PATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPS ENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQF GEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISK IFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILR LFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGL SLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLA DGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMA ALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF

      • 予測される分子量

        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

    • 製品の状態

      Liquid

    • 備考

      Previously labelled as ACADVL. 

    • 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.

      pH: 8.00
      Constituents: 0.0154% DTT, 0.316% Tris HCl, 0.0292% EDTA, 10% Glycerol (glycerin, glycerine), 0.58% Sodium chloride

    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

    • SDS-PAGE - Recombinant Human ACADVL/VLCAD protein (ab98234)
      SDS-PAGE - Recombinant Human ACADVL/VLCAD protein (ab98234)
      15% SDS-PAGE analysis of 3µg ab98234

    プロトコール

    To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.

    Click here to view the general protocols

    参考文献 (0)

    ab98234 を使用した論文を発表された方は、こちらまでお知らせください。データシートに掲載させていただきます。

    ab98234 は論文での使用が確認できていません。

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