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 Mannose-1-phosphate guanyltransferase beta (GMPPB)

  • 中文名稱:
    人GMPPB重組蛋白
  • 貨號:
    CSB-YP896740HU
  • 規格:
  • 來源:
    Yeast
  • 其他:
  • 中文名稱:
    人GMPPB重組蛋白
  • 貨號:
    CSB-EP896740HU
  • 規格:
  • 來源:
    E.coli
  • 其他:
  • 中文名稱:
    人GMPPB重組蛋白
  • 貨號:
    CSB-EP896740HU-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GMPPB重組蛋白
  • 貨號:
    CSB-BP896740HU
  • 規格:
  • 來源:
    Baculovirus
  • 其他:
  • 中文名稱:
    人GMPPB重組蛋白
  • 貨號:
    CSB-MP896740HU
  • 規格:
  • 來源:
    Mammalian cell
  • 其他:

產品詳情

  • 純度:
    >85% (SDS-PAGE)
  • 基因名:
    GMPPB
  • Uniprot No.:
  • 別名:
    GDP mannose pyrophosphorylase B; GDP-mannose pyrophosphorylase B; GMPPB; GMPPB_HUMAN; GTP-mannose-1-phosphate guanylyltransferase beta; KIAA1851; Mannose 1 phosphate guanylyltransferase ; Mannose-1-phosphate guanyltransferase beta
  • 種屬:
    Homo sapiens (Human)
  • 蛋白長度:
    full length protein
  • 表達區域:
    1-360
  • 氨基酸序列
    MKALILVGGY GTRLRPLTLS TPKPLVDFCN KPILLHQVEA LAAAGVDHVI LAVSYMSQVL EKEMKAQEQR LGIRISMSHE EEPLGTAGPL ALARDLLSET ADPFFVLNSD VICDFPFQAM VQFHRHHGQE GSILVTKVEE PSKYGVVVCE ADTGRIHRFV EKPQVFVSNK INAGMYILSP AVLQRIQLQP TSIEKEVFPI MAKEGQLYAM ELQGFWMDIG QPKDFLTGMC LFLQSLRQKQ PERLCSGPGI VGNVLVDPSA RIGQNCSIGP NVSLGPGVVV EDGVCIRRCT VLRDARIRSH SWLESCIVGW RCRVGQWVRM ENVTVLGEDV IVNDELYLNG ASVLPHKSIG ESVPEPRIIM
  • 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:
    Catalyzes the formation of GDP-mannose, an essential precursor of glycan moieties of glycoproteins and glycolipids.
  • 基因功能參考文獻:
    1. Late-onset limb-girdle muscular dystrophy caused by GMPPB mutations. PMID: 28478914
    2. Study finds that the GMPPB mutation spectrum in Chinese patients may differ from that of European populations, with the mutation p.(Arg357His) most frequently found. These mutations may lead to abnormal folding of GMPPB leading to protein aggregates in the cytoplasm rather than an overall loss in protein expression. PMID: 28433477
    3. Patients with GMPPB-CMS have phenotypic features aligned with CMS subtypes harbouring mutations within the early stages of the glycosylation pathway. Additional features shared with the dystroglycanopathies include myopathic features, raised Creatine Kinase levels and variable mild cognitive delay. PMID: 27147698
    4. We observe that c.79G>C (p.D27H) is associated with a mild limb-girdle muscular dystrophy phenotype, whereas c.860G>A (p.R287Q) is associated with a relatively severe congenital muscular dystrophy typically involving brain development. PMID: 26310427
    5. This study found mutations in GMPPB can lead to a wide spectrum of clinical features where deficit in neuromuscular transmission is the major component in a subset of cases. PMID: 26133662
    6. The phenotypic spectrum of GMPPB mutations was expanded to include limb-girdle muscular dystrophies. PMID: 25681410
    7. Work confirms a role for GMPPB defects in alpha-dystroglycanopathy, and suggests that glycosylation may play a role in the neuronal membrane channels or networks involved in the physiology of generalized epilepsy syndromes. PMID: 24780531
    8. Individuals with GMPPB mutations have hypoglycosylated alpha-dystroglycan in muscle. These mutations cause congenital and limb-girdle muscular dystrophies. PMID: 23768512

    顯示更多

    收起更多

  • 相關疾?。?/div>
    Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A14 (MDDGA14); Muscular dystrophy-dystroglycanopathy congenital with mental retardation B14 (MDDGB14); Muscular dystrophy-dystroglycanopathy limb-girdle C14 (MDDGC14)
  • 亞細胞定位:
    Cytoplasm.
  • 蛋白家族:
    Transferase hexapeptide repeat family
  • 數據庫鏈接:

    HGNC: 22932

    OMIM: 615320

    KEGG: hsa:29925

    UniGene: Hs.567488



主站蜘蛛池模板: 亚洲人成网线在线播放| 热re99久久精品国产66热| 熟妇人妻无乱码中文字幕| 中文字幕av无码一区二区三区| 一二三四在线观看免费视频| 蜜臀av综合网| 好紧好爽好深再快点av在线| 精产一二三产区m553| 人妻丰满熟妇aⅴ无码| 久久精品中文无码资源站| 久久久久久人妻毛片a片| 国产亚洲精品久久77777| 国产精品成人99一区无码| 人妻夜夜爽天天爽| 亚洲男人片片在线观看| 无码av高潮喷水无码专区线| 国产精品久久久久久久久久久久人四虎| 欧美午夜精品一区二区三区电影| 亚洲国产成人精品无码区在线秒播| 日产精品中文一区二区三区| 人妻中出受孕 中文字幕在线| 无码人妻一区二区三区在线视频| 亚洲综合日韩久久成人av| 亚洲国产av导航第一福利网| 精精国产xxxx视频在线| 国产亚洲情侣一区二区无| 久久午夜色播影院| 超薄肉色丝袜一区二区| 人人妻人人澡人人爽超污| 亚洲国产成人极品综合| 小鲜肉洗澡时自慰网站xnxx | 蜜臀av色欲a片无人一区 | 天堂在线www资源在线| 亚洲美女被黑人巨大在线播放| 免费中文熟妇在线影片| 少妇做爰免费视频网站| 久久综合97丁香色香蕉| 亚洲成_人网站图片| 无码丰满熟妇| 精品无码一区二区三区爱欲| 免费三级现频在线观看播放|