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

CLN6 Antibody

  • 中文名稱:
    CLN6兔多克隆抗體
  • 貨號:
    CSB-PA030158
  • 規格:
    ¥1090
  • 其他:

產品詳情

  • Uniprot No.:
  • 基因名:
    CLN6
  • 別名:
    CLN6; Ceroid-lipofuscinosis neuronal protein 6; Protein CLN6
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Synthesized peptide derived from the C-terminal region of Human CLN6.
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated
  • 抗體亞型:
    IgG
  • 純化方式:
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    WB, IHC, ELISA
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:500-1:2000
    IHC 1:100-1:300
    ELISA 1:40000
  • 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

  • 基因功能參考文獻:
    1. The CLN6 is not only a molecular entity of the anti-aggregate activity conferred by the ER manipulation using TMalphaBC, but also serves as a potential target of therapeutic interventions. PMID: 28476624
    2. describe the spectrum of clinical and neurophysiologic features associated with mutations of CLN6. PMID: 26115733
    3. study demonstrates the central role of the metal transporter, Zip7, in the aberrant biometal metabolism of CLN6 variants of Neuronal ceroid lipofuscinoses. PMID: 24581221
    4. The study describes the first report in the North of Morocco of the CLN6 p.I154del mutation in 3 patients belonging to a large consanguineous family. PMID: 23180398
    5. our results add CLN6 to the genetic mutations causing teenage-onset progressive myoclonus epilepsy PMID: 22883287
    6. CLN6 and CLN3 mutations trigger distinct processes that converge on a shared pathway, which is responsible for proper subunit c protein turnover and neuronal cell survival. PMID: 21359198
    7. Sequencing of CLN6 will provide a simple diagnostic strategy in this disorder, in which definitive identification usually requires invasive biopsy. PMID: 21549341
    8. Expression studies of three mutations found in CLN6 patients predicted to affect transmembrane domain 3, cytoplasmic loop 2 or result in a truncated membrane protein respectively, is reported. PMID: 20020536
    9. gene mutated in variant late-infantile neuronal ceroid lipofuscinosis (CLN6) and in nclf mutant mice encodes a novel predicted transmembrane protein PMID: 11727201
    10. novel approximately 36-kD CLN6-gene product augments an intriguing set of unrelated membrane-spanning proteins, whose deficiency causes neuronal ceroid lipofuscinosis in mouse and man PMID: 11791207
    11. Eight novel mutations identified in CLN6 in 26 families with late infantile neuronal ceroid lipofuscinosis. PMID: 12815591
    12. ER-resident CLN6 protein lead to lysosomal dysfunctions, which may result in lysosomal accumulation of storage material PMID: 15010453
    13. CLN6 is an ER resident protein, the activity of which, despite this location, must contribute to lysosomal function. PMID: 15265688
    14. These data indicate that CLN6 mutations, in addition to those of CLN8, should be considered a diagnostic alternative in Turkish variant late-infantile neuronal ceroid lipofuscinosis patients. PMID: 15996215
    15. Cholesterol accumulation in lysosomes suggests a homeostasis block as a result of CLN6p deficiency, while dysfunctional endosomal/lysosomal vesicles may act as one of the triggers for apoptosis and cell death. PMID: 16857350
    16. CLN6 maintains its endoplasmic reticulum localization by expressing retention signals present in both the N-terminal cytosolic domain and in the carboxy-proximal transmembrane domains 6 and 7. PMID: 17453415
    17. knockdown of SEL1L [sel-1 suppressor of lin-12-like (Caenorhabditis elegans)], a member of an E3 ubiquitin ligase complex involved in ER protein extraction, rescued significant amounts of Cln6(G123D) and Cln6(M241T) polypeptides. PMID: 18811591
    18. 11 mutations in patients with neuronal ceroid lipofuscinoses, eight of which are novel, were detected in CLN6, all predicting a direct impairing of the putative gene function. PMID: 19135028
    19. three families with CLN6-associated variant late infantile neuronal ceroid lipofuscinosis from Saudi Arabia are described; one had a novel mutation in the CLN6 gene PMID: 19520283

    顯示更多

    收起更多

  • 相關疾病:
    Ceroid lipofuscinosis, neuronal, 6 (CLN6); Ceroid lipofuscinosis, neuronal, 4A (CLN4A)
  • 亞細胞定位:
    Endoplasmic reticulum membrane; Multi-pass membrane protein. Endoplasmic reticulum.
  • 數據庫鏈接:

    HGNC: 2077

    OMIM: 204300

    KEGG: hsa:54982

    STRING: 9606.ENSP00000249806

    UniGene: Hs.584921



主站蜘蛛池模板: 无码三级国产三级在线电影| 无码人妻一区二区三区免费n鬼沢| 欧洲-级毛片内射| 免费无码av片在线观看| 国产在线看片免费观看| 亚洲国产精品无码久久秋霞| 好紧好湿好黄的视频| 奇米影视7777久久精品| 久久久精品人妻一区亚美研究所| 国产av亚洲精品久久久久久| 69堂人成无码免费视频果冻传媒| 亚洲综合区图片小说区| 精品无码黑人又粗又大又长 | 国产成人精品免费视频大全五级| 99爱在线精品视频免费观看| 日本人与黑人做爰视频网站| 18精品久久久无码午夜福利| 久久久这里只有精品10| 欧美白人最猛性xxxxx| 欧美xxxx做受欧美88| 久久久久无码国产精品一区 | 亚洲偷自拍国综合| 精品亚洲成a人在线观看青青 | 亚洲乱码中文字幕综合| 国内精品久久久久久久久齐齐| 偷窥村妇洗澡毛毛多| 久久青青草原精品国产| 色老板精品视频在线观看| 性大毛片视频| 亚洲综合日韩久久成人av| 久久高清超碰av热热久久| 精品人妻无码一区二区三区蜜桃一 | 天天爱天天做天天爽夜夜揉 | 国产成人久久综合第一区| 成人动漫综合网| 欧美熟妇另类久久久久久多毛| 国产在线无码制服丝袜无码| 亚洲色无码中文字幕在线| 高潮毛片无遮挡高清免费| 国语做受对白xxxxx在线| 午夜免费啪视频在线观看区 |