製品の概要

  • 製品名Anti-ACADVL antibody
    ACADVL 一次抗体 製品一覧
  • 製品の詳細
    Goat polyclonal to ACADVL
  • 特異性This antibody is expected to recognize both reported isoforms (NP_000009.1; NP_001029031.1).
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
  • 免疫原

    Synthetic peptide corresponding to N terminal amino acids 50-63 (

    DKSDSHPSDALTRK

    ) of Human ACADVL (NP_000009.1).

  • ポジティブ・コントロール
    • Human heart lysate

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab99490 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 Abreviews 特記事項
WB Use a concentration of 1 - 3 µg/ml. Detects a band of approximately 68 kDa (predicted molecular weight: 68 kDa).

ターゲット情報

  • 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
  • 参照データベース
  • 別名
    • ACAD 6 antibody
    • ACAD6 antibody
    • ACADV_HUMAN antibody
    • Acadvl antibody
    • Acyl CoA dehydrogenase very long chain antibody
    • Acyl Coenzyme A dehydrogenase very long chain antibody
    • LCACD antibody
    • mitochondrial antibody
    • Very long chain specific acyl CoA dehydrogenase antibody
    • Very long chain specific acyl CoA dehydrogenase mitochondrial antibody
    • Very long-chain specific acyl-CoA dehydrogenase antibody
    • VLCAD antibody
    see all

Anti-ACADVL antibody 画像

  • Anti-ACADVL antibody (ab99490) at 2 µg/ml + Human heart lysate (in RIPA buffer) at 35 µg

    Predicted band size : 68 kDa

Anti-ACADVL antibody (ab99490) 使用論文

ab99490 has not yet been referenced specifically in any publications.

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