製品の概要

  • 製品名
  • 製品の詳細
    Mouse polyclonal to HADHB
  • 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

    Recombinant full length Human HADHB protein (NP_000174.1).

  • ポジティブ・コントロール
    • Human liver lysate, A431 cell lysate, and HADHB transfected 293T cell lysate.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab88256 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 µg/ml. Predicted molecular weight: 51 kDa.

ターゲット情報

  • パスウェイ
    Lipid metabolism; fatty acid beta-oxidation.
  • 関連疾患
    Defects in HADHB are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015]. The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all three enzyme activities of the TFP complex.
  • 配列類似性
    Belongs to the thiolase family.
  • 細胞内局在
    Mitochondrion.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 2 enoyl Coenzyme A (CoA) hydratase beta subunit antibody
    • 3 ketoacyl Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein beta subunit antibody
    • 3 ketoacyl Coenzyme A thiolase antibody
    • 3 ketoacyl Coenzyme A thiolase of mitochondrial trifunctional protein beta subunit antibody
    • 3-ketoacyl-CoA thiolase antibody
    • Acetyl CoA acyltransferase antibody
    • Acetyl-CoA acyltransferase antibody
    • Beta ketothiolase antibody
    • Beta-ketothiolase antibody
    • ECHB antibody
    • ECHB_HUMAN antibody
    • HADH antibody
    • Hadhb antibody
    • Hydroxyacyl CoA dehydrogenase/3 ketoacyl CoA thiolase/enoyl CoA hydratase (trifunctional protein), beta subunit antibody
    • Hydroxyacyl Coenzyme A (CoA) dehydrogenase beta subunit antibody
    • Hydroxyacyl Coenzyme A dehydrogenase antibody
    • Hydroxyacyl Coenzyme A dehydrogenase beta subunit antibody
    • Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) beta subunit antibody
    • MGC87480 antibody
    • Mitochondrial trifunctional enzyme beta subunit antibody
    • Mitochondrial trifunctional protein beta subunit antibody
    • MSTP 029 antibody
    • MSTP029 antibody
    • MTPB antibody
    • TP beta antibody
    • TP-beta antibody
    • TPbeta antibody
    • Trifunctional enzyme subunit beta antibody
    • Trifunctional enzyme subunit beta mitochondrial antibody
    • Trifunctional protein antibody
    see all

画像

  • All lanes : Anti-HADHB antibody (ab88256) at 1 µg/ml

    Lane 1 : HADHB transfected 293T cell lysate
    Lane 2 : Untransfected 293T cell lysate

    Lysates/proteins at 25 µg per lane.

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/2500 dilution

    Predicted band size : 51 kDa
    Observed band size : 46 kDa (why is the actual band size different from the predicted?)
  • Anti-HADHB antibody (ab88256) at 1 µg/ml + Human liver lysate at 25 µg

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/2500 dilution

    Predicted band size : 51 kDa
    Observed band size : 46 kDa (why is the actual band size different from the predicted?)
  • Anti-HADHB antibody (ab88256) at 1 µg/ml + A431 cell lysate at 25 µg

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/2500 dilution

    Predicted band size : 51 kDa
    Observed band size : 48 kDa (why is the actual band size different from the predicted?)

プロトコール

参考文献

ab88256 has not yet been referenced specifically in any publications.

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