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MECR Antibody

  • 货号：
    CSB-PA013639GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q9BV79
  • 基因名：
    MECR
  • 别名：
    AI195831 antibody; CGI 63 antibody; FASN2B antibody; Homolog of yeast 2 enoyl thioester reductase antibody; HsNrbf-1 antibody; HsNrbf1 antibody; mecr antibody; MECR_HUMAN antibody; Mitochondrial 2 enoyl thioester reductase antibody; mitochondrial antibody; NRBF 1 antibody; NRBF-1 antibody; NRBF1 antibody; Nuclear receptor binding factor 1 antibody; Nuclear receptor-binding factor 1 antibody; OTTMUSP00000009996 antibody; RP23-13A13.1 antibody; Trans 2 enoyl CoA reductase; mitochondrial antibody; Trans-2-enoyl-CoA reductase antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human MECR
  • 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：
    Catalyzes the NADPH-dependent reduction of trans-2-enoyl thioesters in mitochondrial fatty acid synthesis (fatty acid synthesis type II). Fatty acid chain elongation in mitochondria uses acyl carrier protein (ACP) as an acyl group carrier, but the enzyme accepts both ACP and CoA thioesters as substrates in vitro. Displays a preference for medium-chain over short- and long-chain substrates. May provide the octanoyl chain used for lipoic acid biosynthesis, regulating protein lipoylation and mitochondrial respiratory activity particularly in Purkinje cells.
  • 基因功能参考文献：
    1. MECR is involved in mitochondrial fatty acid synthesis, and overexpression of this gene increases peroxisome proliferator-activated receptor alpha (PPARalpha) activity. PMID: 27845578
    2. results suggest that mutations in MECR cause a distinct human disorder of the mitochondrial fatty acid synthesis pathway; the observation of decreased lipoylation raises the possibility of a potential therapeutic strategy PMID: 27817865
    3. overexpression of MECR, the last step in the mitochondrial fatty acid synthesis (mtFASII) pathway, causes modulation of gene expression through the PPAR pathway. PMID: 24161390
    4. The crystal structure of this dimeric enzyme (at 2.4 A resolution) suggests that the binding site for the recognition helix of the acyl carrier protein is in a groove between the two adjacent monomers. PMID: 18479707
  • 相关疾病：
    Dystonia, childhood-onset, with optic atrophy and basal ganglia abnormalities (DYTOABG)
  • 亚细胞定位：
    [Isoform 1]: Mitochondrion.; [Isoform 2]: Cytoplasm. Nucleus.
  • 蛋白家族：
    Zinc-containing alcohol dehydrogenase family, Quinone oxidoreductase subfamily
  • 组织特异性：
    Highly expressed in skeletal and heart muscle. Expressed at lower level in placenta, liver, kidney and pancreas. Weakly or not expressed in lung.
  • 数据库链接：

    HGNC: 19691

    OMIM: 608205

    KEGG: hsa:51102

    STRING: 9606.ENSP00000263702

    UniGene: Hs.183646