Anti-HADHB抗体(ab10093)

概述

  • 产品名称Anti-HADHB抗体
    参阅全部 HADHB 一抗
  • 描述
    山羊多克隆抗体to HADHB
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

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

  • 阳性对照
    • Human brain and Jurkat extracts.
  • 常规说明


    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.

性能

相关产品

应用

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.

应用 Ab评论 说明
WB Use at an assay dependent concentration. Can be blocked with Human HADHB peptide (ab23180).

靶标

  • 通路Lipid metabolism; fatty acid beta-oxidation.
  • 疾病相关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.
  • 序列相似性Belongs to the thiolase family.
  • 细胞定位Mitochondrion.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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

Anti-HADHB antibody (ab10093)参考文献

ab10093 has not yet been referenced specifically in any publications.

Product Wall

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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提交于 Sep 04 2009

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