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

DOK7 Antibody, Biotin conjugated

  • 中文名稱:
    DOK7兔多克隆抗體, Biotin偶聯(lián)
  • 貨號:
    CSB-PA623281LD01HU
  • 規(guī)格:
    ¥880
  • 其他:

產(chǎn)品詳情

  • 產(chǎn)品名稱:
    Rabbit anti-Homo sapiens (Human) DOK7 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    DOK7
  • 別名:
    Docking protein 7 antibody; DOK 7 antibody; DOK7 antibody; DOK7_HUMAN antibody; Downstream of tyrosine kinase 7 antibody; Protein Dok-7 antibody
  • 宿主:
    Rabbit
  • 反應(yīng)種屬:
    Human
  • 免疫原:
    Recombinant Human Protein Dok-7 protein (416-504AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標(biāo)記方式:
    Biotin
  • 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(chǎn)品提供形式:
    Liquid
  • 應(yīng)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(chǎn)品評價(jià)

靶點(diǎn)詳情

  • 功能:
    Probable muscle-intrinsic activator of MUSK that plays an essential role in neuromuscular synaptogenesis. Acts in aneural activation of MUSK and subsequent acetylcholine receptor (AchR) clustering in myotubes. Induces autophosphorylation of MUSK.
  • 基因功能參考文獻(xiàn):
    1. Repression of Dok7 expression via DNMT1 mediated DNA methylation promotes glioma cell proliferation. PMID: 29990858
    2. Silencing DNMT3A inhibits proliferation and invasion in ESCC cells by inducing demethylation of DOK7. PMID: 28343076
    3. DOK7 was reduced in lung cancer and reduced DOK7 expression was associated with poorer survival.DOK7 isoform 1 plays an inhibitory role on the proliferation and migration of lung cancer cells. PMID: 28393246
    4. Individuals with DOK7 congenital myasthenic syndrome displayed stridor and feeding difficulties at birth or progressive weakness despite normal milestones in infancy pointing to a diagnosis and should lead to neurophysiological and genetic investigation PMID: 23831158
    5. this study demonistrated that Salbutamol is an effective treatment in patient wity congenital myasthenic syndrome due to DOK7 mutation. PMID: 23790237
    6. DOK7 limb-girdle myasthenic syndrome can mimick congenital muscular dystrophy. PMID: 22884442
    7. Hypermethylation of DOK7 occurs years before tumor diagnosis, suggesting a role as a powerful epigenetic blood-based biomarker as well as providing insights into breast cancer pathogenesis PMID: 23054610
    8. In contrast to AChR deficiency due to epsilon subunit mutations, onset of DOK7 CMS tends to be later--ages two to three years--and in DOK7 CMS eye movements are usually spared and anticholinesterases can exacerbate the weakness PMID: 23278577
    9. The DOK7 gene is highly polymorphic, and within these many variants, a spectrum of mutations that can underlie DOK7 Congenital myasthenic syndromes that will inform in managing this disorder, were defined. PMID: 22661499
    10. Sequencing of DOK-7 in seronegative myasthenia gravis patients reveals no mutations. PMID: 21305573
    11. 6 CMS patients with DOK7 mutations had congenital stridor, bilateral vocal cord palsy and difficulty with feeding PMID: 20554332
    12. This study demonistreated that DOK7 mutation casused congenital myasthenic syndrome in French Canadians. PMID: 20610155
    13. these findings demonstrate that missense mutations in MUSK can result in a severe form of congenital myasthenic syndrome and indicate that the inability of MuSK mutants to interact with Dok-7. PMID: 20371544
    14. We report clinical, morphological and molecular data on 15 congenital myasthenic syndrome patients with mutations in DOK7; characterization of this distinct phenotype is essential to provide clues for targeted genetic screening and therapy PMID: 20012313
    15. The crystal structure of the Dok7 PH-PTB domains in complex with a phosphopeptide representing the Dok7-binding site on MuSK, is presented. PMID: 20603078
    16. Dok-7 is essential for neuromuscular synaptogenesis through its interaction with MuSK PMID: 16794080
    17. findings showed that recessive inheritance of mutations in Dok-7, which result in a defective structure of the neuromuscular junction, is a cause of congenital myasthenic syndromes with proximal muscle weakness PMID: 16917026
    18. study of patients with congenital myasthenic syndromes with mutations in DOK7; none with DOK7 mutations had tubular aggregates in muscle biopsy, implying 'limb-girdle myasthenia with tubular aggregates' may be distinct from CMS caused by DOK7 mutations PMID: 17439981
    19. considerable phenotypic variability associated with congenital myasthenic syndrome due to DOK7 mutations PMID: 18161030
    20. the COOH-terminal NES and Src homology 2 target motifs play key roles in Dok-7/MuSK signaling for neuromuscular synaptogenesis. PMID: 18165682
    21. Dok-7 is essential for not only the size but also the structural integrity of the EP. The structural alterations at the EPs cause the reduced safety margin of neuromuscular transmission. PMID: 18626973
    22. Dok-7 activates the muscle receptor kinase MuSK and shapes synapse formation. PMID: 19244212
    23. whereas incomplete loss of DOK7 function may cause congenital myasthenia, more severe loss of function can result in a lethal fetal akinesia phenotype PMID: 19261599

    顯示更多

    收起更多

  • 相關(guān)疾病:
    Myasthenic syndrome, congenital, 10 (CMS10)
  • 亞細(xì)胞定位:
    Cell membrane; Peripheral membrane protein. Cell junction, synapse.
  • 組織特異性:
    Preferentially expressed in skeletal muscle and heart. Present in thigh muscle, diaphragm and heart but not in the liver or spleen (at protein level).
  • 數(shù)據(jù)庫鏈接:

    HGNC: 26594

    OMIM: 254300

    KEGG: hsa:285489

    STRING: 9606.ENSP00000344432

    UniGene: Hs.122110



主站蜘蛛池模板: 国产精品无码a∨精品影院app| 国产午夜片无码区在线播放| 色综合久久无码中文字幕 | 草色噜噜噜av在线观看香蕉| 精品人妻无码一区二区三区换脸| 日韩精品无码成人专区| 日本少妇浓毛bbwbbwbbw| 亚洲老熟女性亚洲| 久久精品国产大片免费观看| 国产成人一区二区三区在线观看 | 蜜桃av色偷偷av老熟女| 东京热毛片无码dvd一二三区| 精品国产午夜福利精品推荐| 综合久久给合久久狠狠狠97色| 少妇伦子伦情品无吗| 国产亚洲精品久久久久动漫| 天堂va欧美ⅴa亚洲va在线| 国产亚洲产品影市在线产品| 99re6在线视频精品免费| 精品高朝久久久久9999| 国产精品99久久久久久宅男小说| 亚欧免费无码aⅴ在线观看| 狠狠干狠狠爱| 婷婷色香五月综合缴缴情| 亚洲国产婷婷综合在线精品| 中文字幕精品久久久久人妻红杏1| 国产亲子乱弄免费视频| 92国产精品午夜免费福利视频| 国产成人无码激情视频| 无码无套少妇毛多69xxx| 色婷婷av一区二区三区之红樱桃| 老司机免费的精品视频| 欧美精品一区二区精品久久| 亚洲国产成人一区二区精品区 | 国产人妻丰满熟妇嗷嗷叫| 骚女人干起来舒服视频在线| 国产真实乱对白精彩久久 | 日韩精品人妻系列无码专区免费 | 熟女俱乐部五十路六十路av| 亚洲aⅴ天堂av天堂无码app| 东京热久久综合久久88|