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: 亚洲愉拍99热成人精品| 美女被啪到深处抽搐视频| 亚洲精品午夜一区人人爽| 人人爽人人爽人人爽| 国産精品久久久久久久| 丰满岳乱妇一区二区三区| 亚洲人成人无码www| 欧洲国产精品无码专区影院| 亚洲精品无码国产片| 免费无码成人片| 欧美国产日韩a在线观看| 亚洲 日韩 国产 有码 不卡 | 亚洲经典千人经典日产| 欧美18精品久久久无码午夜福利| 国产欧色美视频综合二区| 亚洲午夜精品久久久久久人妖| 日韩网红少妇无码视频香港| 色悠久久久久综合网香蕉| 久久久一本精品99久久精品66直播 | 女同性av片在线观看免费网站| 日本少妇三级hd激情在线观看| 国产v亚洲v天堂无码| 午夜精品久久久久久中宇| 国产成人一区二区精品视频| 色六月婷婷亚洲婷婷六月| 国产综合色在线精品| 亚洲精品国产品国语在线| 亚洲免费人成视频观看| 任我橹这里只有精品 在线视频 | 影音先锋亚洲成aⅴ人在| 国产亚洲美女精品久久久| 国精品午夜福利视频| 狠狠色噜噜狠狠狠狠777米奇小说| 苍井空浴缸大战猛男120分钟| 亚洲第一极品精品无码| 香蕉eeww99国产精选免费| 午夜在线观看免费线无码视频| 人妻被按摩师玩弄到潮喷| 国语自产精品视频在 视频| 亚洲精品一区二区三区中文字幕| 97午夜理论片在线影院|