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, HRP conjugated

PCCB Antibody, HRP conjugated

  • 中文名稱：
    PCCB兔多克隆抗體, HRP偶聯
  • 貨號：
    CSB-PA017523LB01HU
  • 規格：
    ￥880
  • 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：
    HRP
  • 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
  • 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： 色妞色视频一区二区三区四区| 97人洗澡人人澡人人爽人人模 | 中文字幕有码无码av| 国产精品人人做人人爽人人添| 成人精品视频99在线观看免费| 日本少妇裸体做爰高潮片 | 天干天干天啪啪夜爽爽99| 国产日产精品久久快鸭的功能介绍| 亚洲精品色播一区二区| 国产在线精品欧美日韩电影| 欧美欧洲成本大片免费| 66亚洲一卡2卡新区成片发布| 爱做久久久久久| 精品人妻无码区在线视频| 人妻熟女一区二区aⅴ图片| 被窝的午夜无码福利专区| 无码喷水一区二区浪潮av| 成视频年人黄网站免费视频| 亚洲国产成人精品女人久久久| 午夜精品久久久久久久爽| 欧美交a欧美精品喷水| 欧美综合乱图图区乱图图区| 亚洲精品乱码久久久久蜜桃| 最爽爱爱高潮免费视频| 国产成人一区二区三区别| 欧美xxxx狂喷水| 在线观看国产精品va| 精品偷拍一区二区三区在线看| 男女做爰猛烈叫床视频免费| 天天躁日日躁狠狠躁| 天堂8а√中文在线官网| 337p日本欧洲亚洲大胆人人| 成人性做爰aaa片免费看不忠| 国产三级久久久精品麻豆三级 | aaa少妇高潮大片免费看| 亚洲经典国产精华液单品榜| 日本乱人伦aⅴ精品| 久久不见久久见免费视频7| 麻豆国产97在线 | 欧美| 国内熟女啪啪自拍| 小草社区视频在线观看|