在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

  1. Home
  2. 抗體
  3. 多克隆抗體
  4. PCCB Antibody

PCCB Antibody

  • 中文名稱：
    PCCB兔多克隆抗體
  • 貨號：
    CSB-PA017523LA01HU
  • 規格：
    ￥440
  • 圖片：
    • 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：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) PCCB Polyclonal antibody
  • Uniprot No.：
  • 基因名：
  • 別名：
    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
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Propionyl-CoA carboxylase beta chain, mitochondrial protein (200-314AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated

    本頁面中的產品，PCCB Antibody (CSB-PA017523LA01HU)，的標記方式是Non-conjugated。對于PCCB Antibody，我們還提供其他標記。見下表：

    可提供標記
    標記方式 貨號 產品名稱 應用
    HRP CSB-PA017523LB01HU PCCB Antibody, HRP conjugated ELISA
    FITC CSB-PA017523LC01HU PCCB Antibody, FITC conjugated
    Biotin CSB-PA017523LD01HU PCCB Antibody, Biotin conjugated ELISA
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, WB, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:200-1:500
  • Protocols：
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    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.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 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.
  • 基因功能參考文獻：
    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

    顯示更多

    收起更多

  • 相關疾病：
    Propionic acidemia type II (PA-2)
  • 亞細胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    AccD/PCCB family
  • 數據庫鏈接：

    HGNC: 8654

    OMIM: 232050

    KEGG: hsa:5096

    STRING: 9606.ENSP00000419027

    UniGene: Hs.63788



主站蜘蛛池模板： 亚洲精品国产自在现线看| 天干天干天啪啪夜爽爽av| 日韩无码电影| 国内久久婷婷五月综合色| 欧美日本特级婬片视频| 香蕉久久人人爽人人爽人人片av| 国产精品久久久久久妇女6080| 狠狠躁夜夜躁人人躁婷婷 | 国产精品人人妻人人爽| 精品麻豆丝袜高跟鞋av| 中国精品无码免费专区午夜| 国产成人综合久久免费导航| 久久久久波多野结衣高潮| 亚洲精品成人a在线观看| 好紧好爽免费午夜视频| 国产亚洲曝欧美不卡精品| 成 年 人 黄 色 大 片大 全| 免费国产va在线观看| 免费国产成人高清在线视频| 成人奭片免费观看| 人妻去按摩店被黑人按中出| www插插插无码视频网站| 欧美人与动人物姣配xxxx| 色噜噜噜亚洲男人的天堂| 一二区成人影院电影网| 国产精品无码一区二区桃花视频| 综合在线视频精品专区| 亚洲综合久久无码色噜噜| 国产午夜精品一区二区三区不卡| 少妇大叫太大太粗太爽了a片小说| 精品亚洲成a人在线观看青青| 97av麻豆蜜桃一区二区| 肉岳疯狂69式激情的高潮 | 国产精品一区二区久久| 麻豆精品人妻一区二区三区蜜桃 | 亚洲中文字幕av每天更新 | 亚洲国产精品综合久久20| 欧美性xxxx极品少妇| 亚洲成本人无码薄码区| 无码人妻丰满熟妇区bbbbxxxx| 伊人色综合久久天天五月婷|