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PCCB Antibody

  • 货号：
    CSB-PA017523LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: Jurkat whole cell lysate
      All lanes: PCCB antibody at 7.7µg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 59, 61 kDa
      Observed band size: 59 kDa
    • IHC image of CSB-PA017523LA01HU diluted at 1:300 and staining in paraffin-embedded human pancreatic cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • IHC image of CSB-PA017523LA01HU diluted at 1:300 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) PCCB Polyclonal antibody
  • Uniprot No.：
    P05166
  • 基因名：
    PCCB
  • 别名：
    DKFZp451E113 antibody; PCCase subunit beta antibody; pccB antibody; PCCB_HUMAN antibody; pccBC Complementation group antibody; Propanoyl CoA:carbon dioxide ligase subunit beta antibody; Propanoyl-CoA:carbon dioxide ligase subunit beta antibody; Propionyl CoA carboxylase beta chain; mitochondrial antibody; propionyl Coenzyme A carboxylase; beta polypeptide antibody; Propionyl-CoA carboxylase ; beta subunit antibody; Propionyl-CoA carboxylase beta chain; mitochondrial antibody; R74805 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Propionyl-CoA carboxylase beta chain, mitochondrial protein (200-314AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，PCCB Antibody (CSB-PA017523LA01HU)，的标记方式是Non-conjugated。对于PCCB 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-PA017523LB01HU PCCB Antibody, HRP conjugated ELISA
    FITC CSB-PA017523LC01HU PCCB Antibody, FITC conjugated
    Biotin CSB-PA017523LD01HU PCCB 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, WB, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:200-1:500
  • 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：
    This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA. Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate. Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA.
  • 基因功能参考文献：
    1. Recurrent lactic acid elevations were present in six out of the eight patients. Five of the eight patients were diagnosed with Autism Spectrum Disorder, four of them had pathogenic variants in PCCB PMID: 27825584
    2. The majority of patients had mutations in the PCCA gene (18/25). A total of 26 mutations were noted: 20 in the PCCA gene and 6 in PCCB gene. Seventeen mutations were novel (14 in PCCA and 3 in PCCB). PMID: 27227689
    3. The c.-4156_183+3713del mutation is the first known large deletion that affects the PCCB gene functions. PMID: 24863100
    4. mutations affect homomeric and heteromeric assembly of Propionyl-CoA Carboxylase PMID: 11749052
    5. Structure-function analysis of a range of isolated PCCB subunit mutants has elucidated the complex relationship between genotype and phenotype in propionic acidemia. PMID: 12007220
    6. Work showed 15 novel PCCB gene mutations and phenotype-genotype correlations for the prediction of the metabolic outcome and for the implementation of treatments tailored to each PA patient. PMID: 12559849
    7. pathogenicity of R67S, R165Q and G112D mutations in PCCB gene in propionic acidemia PMID: 12757933

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  • 相关疾病：
    Propionic acidemia type II (PA-2)
  • 亚细胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    AccD/PCCB family
  • 数据库链接：

    HGNC: 8654

    OMIM: 232050

    KEGG: hsa:5096

    STRING: 9606.ENSP00000419027

    UniGene: Hs.63788