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

Recombinant Human Propionyl-CoA carboxylase beta chain, mitochondrial (PCCB)

  • 中文名稱:
    人PCCB重組蛋白
  • 貨號:
    CSB-YP017523HU
  • 規格:
  • 來源:
    Yeast
  • 其他:
  • 中文名稱:
    人PCCB重組蛋白
  • 貨號:
    CSB-EP017523HU-B
  • 規格:
  • 來源:
    E.coli
  • 共軛:
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他:
  • 中文名稱:
    人PCCB重組蛋白
  • 貨號:
    CSB-BP017523HU
  • 規格:
  • 來源:
    Baculovirus
  • 其他:
  • 中文名稱:
    人PCCB重組蛋白
  • 貨號:
    CSB-MP017523HU
  • 規格:
  • 來源:
    Mammalian cell
  • 其他:

產品詳情

  • 純度:
    >85% (SDS-PAGE)
  • 基因名:
  • Uniprot No.:
  • 別名:
    DKFZp451E113; PCCase subunit beta; pccB; PCCB_HUMAN; pccBC Complementation group; Propanoyl CoA:carbon dioxide ligase subunit beta; Propanoyl-CoA:carbon dioxide ligase subunit beta; Propionyl CoA carboxylase beta chain; mitochondrial ; propionyl Coenzyme A carboxylase; beta polypeptide; Propionyl-CoA carboxylase ; beta subunit ; Propionyl-CoA carboxylase beta chain; mitochondrial; R74805
  • 種屬:
    Homo sapiens (Human)
  • 蛋白長度:
    Full Length of Mature Protein
  • 表達區域:
    29-539
  • 氨基酸序列
    CS QATSVNERIE NKRRTALLGG GQRRIDAQHK RGKLTARERI SLLLDPGSFV ESDMFVEHRC ADFGMAADKN KFPGDSVVTG RGRINGRLVY VFSQDFTVFG GSLSGAHAQK ICKIMDQAIT VGAPVIGLND SGGARIQEGV ESLAGYADIF LRNVTASGVI PQISLIMGPC AGGAVYSPAL TDFTFMVKDT SYLFITGPDV VKSVTNEDVT QEELGGAKTH TTMSGVAHRA FENDVDALCN LRDFFNYLPL SSQDPAPVRE CHDPSDRLVP ELDTIVPLES TKAYNMVDII HSVVDEREFF EIMPNYAKNI IVGFARMNGR TVGIVGNQPK VASGCLDINS SVKGARFVRF CDAFNIPLIT FVDVPGFLPG TAQEYGGIIR HGAKLLYAFA EATVPKVTVI TRKAYGGAYD VMSSKHLCGD TNYAWPTAEI AVMGAKGAVE IIFKGHENVE AAQAEYIEKF ANPFPAAVRG FVDDIIQPSS TRARICCDLD VLASKKVQRP WRKHANIPL
  • 蛋白標簽:
    Tag?type?will?be?determined?during?the?manufacturing?process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 產品提供形式:
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 復溶:
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 儲存條件:
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質期:
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期:
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項:
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet :
    Please contact us to get it.

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: 又色又爽又黄的视频网站| 永久免费无码av在线网站| 亚洲欧洲日本综合aⅴ在线| 久久成人a毛片免费观看网站| 狠狠色狠狠色综合日日不卡| 亚洲综合色区无码专区| 国内精品无码一区二区三区| 无人区一线二线三线乱码| 野外亲子乱子伦视频丶 | 欧美日韩在线亚洲综合国产人| 国产欧美日韩| 青青青国产在线观看资源| 国产又色又爽又黄的在线观看| 夜夜高潮夜夜爽国产伦精品| 欧性猛交ⅹxxx乱大交| 国内精品久久久久影院中文字幕| 午夜一区二区亚洲福利| 国产精品亚洲一区二区三区在线| 少妇无码av无码专区线| 精品国内自产拍在线观看| 精品国产一区二区三区久久久狼| 国内精品久久久久久中文字幕| 亚洲综合色噜噜狠狠网站超清| 国产精品av一区二区三区不卡蜜| 丰满的少妇xxxxx青青青| 18禁成年免费无码国产| 亚洲韩国日本高清一区| 色偷偷偷久久伊人大杳蕉| 蜜臀av在线观看| 老妇肥熟凸凹丰满刺激| 亚洲中文欧美在线视频| 国产亚洲曝欧美不卡精品| 日韩熟女精品一区二区三区 | 欧美gv在线观看| 色丁狠狠桃花久久综合网| 天堂无码人妻精品av一区| 无码av大香线蕉| 成人国产精品无码网站| 夜夜揉揉日日人人| 国偷自产av一区二区三区| 久久精品亚洲男人的天堂|