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HADHB Antibody

  • 货号：
    CSB-PA03035A0Rb
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human prostate tissue using CSB-PA03035A0Rb at dilution of 1:50
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) HADHB Polyclonal antibody
  • Uniprot No.：
    P55084
  • 基因名：
    HADHB
  • 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
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Trifunctional enzyme subunit beta, mitochondrial protein (35-283AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，HADHB Antibody (CSB-PA03035A0Rb)，的标记方式是Non-conjugated。对于HADHB Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA03035B0Rb HADHB Antibody, HRP conjugated ELISA
    FITC CSB-PA03035C0Rb HADHB Antibody, FITC conjugated
    Biotin CSB-PA03035D0Rb HADHB Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities, while the trifunctional enzyme subunit beta/HADHB described here bears the 3-ketoacyl-CoA thiolase activity.
  • 基因功能参考文献：
    1. Mutations in HADHB, which encodes the beta-subunit of mitochondrial trifunctional protein, cause infantile onset hypoparathyroidism and peripheral polyneuropathy. PMID: 24664533
    2. Heterozygous mutation in HADHB gene cause early-onset axonal axonal Charcot-Marie-tooth disease. PMID: 24314034
    3. mutational analysis of the HADHB gene, which encodes long-chain 3-ketoacyl-CoA thiolase, identified compound heterozygous mutations of c.520C>T (p.R141C) and c.1331G>A (p.R411K) in a case of mitochondrial trifunctional protein deficiency PMID: 22000755
    4. The present findings showed that all missense mutations in HADHB were disease-causing. PMID: 19699128
    5. HADHB trifunctional enzyme, human renin, and poly(C)-binding protein are novel renin mRNA-binding proteins that target a cis-element in the 3'-UTR of renin mRNA and regulate renin production PMID: 12933794
    6. The common disease causing mutation of G1528C in MTP gene in caucasian in probably not a common mutation in Chinese Han people in Beijing. PMID: 17199921

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  • 相关疾病：
    Mitochondrial trifunctional protein deficiency (MTPD)
  • 亚细胞定位：
    Mitochondrion. Mitochondrion inner membrane. Mitochondrion outer membrane. Endoplasmic reticulum.
  • 蛋白家族：
    Thiolase family
  • 数据库链接：

    HGNC: 4803

    OMIM: 143450

    KEGG: hsa:3032

    STRING: 9606.ENSP00000325136

    UniGene: Hs.515848