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EHHADH Antibody

  • 货号：
    CSB-PA003717
  • 规格：
    ￥880
  • 图片：
    • Western Blot analysis of A549 cells using PBFE Polyclonal Antibody
    • Western Blot analysis of Jurkat K562 NIH-3T3 cells using PBFE Polyclonal Antibody
  • 其他：

产品详情

  • Uniprot No.：
    Q08426
  • 基因名：
  • 别名：
    3 hydroxyacyl CoA dehydrogenase antibody; 3,2 trans enoyl CoA isomerase antibody; 3-hydroxyacyl-CoA dehydrogenase antibody; ECHD antibody; ECHP_HUMAN antibody; EHHADH antibody; Enoyl Coenzyme A antibody; Enoyl Coenzyme A; hydratase/3 hydroxyacyl Coenzyme A dehydrogenase antibody; L 3 hydroxyacyl CoA dehydrogenase antibody; L bifunctional protein antibody; L bifunctional protein; peroxisomal antibody; L PBE antibody; LBFP antibody; LBP antibody; MGC120586 antibody; MS730 antibody; PBE antibody; PBFE antibody; Peroxisomal bifunctional enzyme antibody; Peroxisomal enoyl CoA hydratase antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Rat
  • 免疫原：
    Synthesized peptide derived from the Internal region of Human PBFE.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB, IHC, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    IHC 1:100-1:300
    ELISA 1:10000
  • 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：
    Peroxisomal trifunctional enzyme possessing 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and delta 3, delta 2-enoyl-CoA isomerase activities. Catalyzes two of the four reactions of the long straight chain fatty acids peroxisomal beta-oxidation pathway. Optimal isomerase for 2,5 double bonds into 3,5 form isomerization in a range of enoyl-CoA species (Probable). Also able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species. With HSD17B4, catalyzes the hydration of trans-2-enoyl-CoA and the dehydrogenation of 3-hydroxyacyl-CoA, but with opposite chiral specificity. Regulates the amount of medium-chain dicarboxylic fatty acids which are essential regulators of all fatty acid oxidation pathways. Also involved in the degradation of long-chain dicarboxylic acids through peroxisomal beta-oxidation.
  • 基因功能参考文献：
    1. Mistargeting of peroxisomal EHHADH disrupts mitochondrial metabolism and leads to renal Fanconi's syndrome; this indicates a central role of mitochondria in proximal tubular function. PMID: 24401050
  • 相关疾病：
    Fanconi renotubular syndrome 3 (FRTS3)
  • 亚细胞定位：
    Peroxisome.
  • 蛋白家族：
    Enoyl-CoA hydratase/isomerase family; 3-hydroxyacyl-CoA dehydrogenase family
  • 组织特异性：
    Liver and kidney. Strongly expressed in the terminal segments of the proximal tubule. Lower amounts seen in the brain.
  • 数据库链接：

    HGNC: 3247

    OMIM: 607037

    KEGG: hsa:1962

    STRING: 9606.ENSP00000231887

    UniGene: Hs.429879