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MCCC2 Antibody

  • 货号：
    CSB-PA013573GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q9HCC0
  • 基因名：
    MCCC2
  • 别名：
    MCCC2 antibody; MCCBMethylcrotonoyl-CoA carboxylase beta chain antibody; mitochondrial antibody; MCCase subunit beta antibody; EC 6.4.1.4 antibody; 3-methylcrotonyl-CoA carboxylase 2 antibody; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit antibody; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human MCCC2
  • 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：
    Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.
  • 基因功能参考文献：
    1. Novel mutation in MCCC2 gene was identified in Chinese population. PMID: 25382614
    2. Mutation in 3-methylcrotonyl CoA carboxylase 2 gene is associated with 3-methylcrotonyl-CoA carboxylase deficiency. PMID: 22264772
    3. The amino-termini containing 20 amino acids (MCCbeta) were both necessary and sufficient for targeting. Structural requirements for mitochondrial import were defined by site-directed mutagenesis. PMID: 16023992
    4. The Kd value of soraphen A for the BC domains of human ACC1 and ACC2 is 1 nM. This high binding affinity is mainly due to the extensive interactions between soraphen A and the human biotin carboxylase domain PMID: 17876819
  • 相关疾病：
    3-methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D)
  • 亚细胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    AccD/PCCB family
  • 数据库链接：

    HGNC: 6937

    OMIM: 210210

    KEGG: hsa:64087

    STRING: 9606.ENSP00000343657

    UniGene: Hs.604789