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HIBCH Antibody

  • 货号：
    CSB-PA010347GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q6NVY1
  • 基因名：
    HIBCH
  • 别名：
    3 hydroxyisobutyryl Coenzyme A hydrolase antibody; 3 hydroxyisobutyryl Coenzyme A hydrolase; mitochondrial antibody; 3-hydroxyisobutyryl-CoA hydrolase antibody; 3-hydroxyisobutyryl-coenzyme A hydrolase antibody; BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE antibody; HIB CoA hydrolase antibody; HIB-CoA hydrolase antibody; HIBCH antibody; HIBCH_HUMAN antibody; HIBCoA hydrolase antibody; HIBYL CoA H antibody; HIBYL CoAH antibody; HIBYL-CoA-H antibody; HIBYLCoA H antibody; HIBYLCoAH antibody; mitochondrial antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human HIBCH
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 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：
    Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.
  • 基因功能参考文献：
    1. Novel (founder) mutation in HIBCH that causes a mild phenotype, allowing survival into adulthood. PMID: 27400804
    2. Polymorphism in HIBCH is associated with HIBCH deficiency. PMID: 27132595
    3. findings demonstrated a novel homozygous pathogenic missense mutation c.950G
    4. Molecular analysis in both patients uncovered mutations in the HIBCH gene, including one missense mutation in a conserved part of the protein and two mutations affecting splicing. PMID: 17160907
  • 相关疾病：
    3-hydroxyisobutryl-CoA hydrolase deficiency (HIBCHD)
  • 亚细胞定位：
    Mitochondrion.
  • 蛋白家族：
    Enoyl-CoA hydratase/isomerase family
  • 组织特异性：
    Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung.
  • 数据库链接：

    HGNC: 4908

    OMIM: 250620

    KEGG: hsa:26275

    STRING: 9606.ENSP00000352706

    UniGene: Hs.656685