製品の概要

  • 製品名
  • 製品の詳細
    Goat 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

    Synthetic peptide: TILTYPFKNLPT, corresponding to N terminal amino acids 2-13 of Human HADHB.

  • ポジティブ・コントロール
    • Human brain and Jurkat extracts.
  • 特記事項


    The HADHA and HADHB genes encode the alpha and beta subunits of the mitochondrial trifunctional protein, respectively. The heterocomplex contains 4 alpha and 4 beta subunits and catalyzes 3 steps in the beta-oxidation of fatty acids, including the long-chain 3-hydroxyl-CoA dehydrogenase step. Deficiency of this complex causes sudden unexplained infant death, Reye-like syndrome, cardiomyopathy, or skeletal myopathy. Defects in HADHB are a cause of a variety of diseases such as hypoglycemia, hyperammonemia, mild liver dysfunction and 3-hydroxydicarboxylic aciduria.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab10093 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 at an assay dependent concentration. Can be blocked with Human HADHB peptide (ab23180).

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

プロトコール

参考文献

ab10093 has not yet been referenced specifically in any publications.

レビューと Q&A

Abcam guarantees this product to work in the species/application used in this Abreview.
Application
Western blot
Sample
Human Cell lysate - whole cell (Huh 7.5 cells)
Loading amount
40 µg
Specification
Huh 7.5 cells
Gel Running Conditions
Reduced Denaturing (12.5% gel)
Blocking step
(agent) for 20 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: 4°C
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投稿 Sep 04 2009

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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