製品の概要

製品の詳細

  • 由来
    Recombinant
  • 由来
    Wheat germ
  • アミノ酸配列
    • アクセッション番号
    • 生物種
      Human
    • 配列
      MAARLLRGSLRVLGGHRAPRQLPAARCSHSGGEERLETPSAKKLTDIGIR RIFSPEHDIFRKSVRKFFQEEVIPHHSEWEKAGEVSREVWEKAGKQGLLG VNIAEHLGGIGGDLYSAAIVWEEQAYSNCSGPGFSIHSGIVMSYITNHGS EEQIKHFIPQMTAGKCIGAIAMTEPGAGSDLQGIKTNAKKDGSDWILNGS KVFISNGSLSDVVIVVAVTNHEAPSPAHGISLFLVENGMKGFIKGRKLHK MGLKAQDTAELFFEDIRLPASALLGEENKGFYYIMKELPQERLLIADVAI SASEFMFEETRNYVKQRKAFGKTVAHLQTVQHKLAELKTHICVTRAFVDN CLQLHEAKRLDSATACMAKYWASELQNSVAYDCVQLHGGWGYMWEYPIAK AYVDARVQPIYGGTNEIMKELIAREIVFDK
    • 分子量
      73 kDa including tags
    • 領域
      1 to 430

関連製品

特性

Our Abpromise guarantee covers the use of ab114591 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

    ELISA

    SDS-PAGE

    Western blot

  • 製品の状態
    Liquid
  • 備考
    Protein concentration is above or equal to 0.05 µg/ul. Best use within three months from the date of receipt of this protein.
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

関連情報

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

画像

  • 12.5% SDS-PAGE showing ab114591 at approximately 73.41kDa stained with Coomassie Blue.

参考文献

ab114591 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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