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

HPS6 Antibody, FITC conjugated

  • 中文名稱:
    HPS6兔多克隆抗體, FITC偶聯
  • 貨號:
    CSB-PA803158LC01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) HPS6 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    HPS6
  • 別名:
    BLOC2S3 antibody; FLJ22501 antibody; Hermansky Pudlak syndrome 6 protein antibody; Hermansky-Pudlak syndrome 6 protein antibody; HPS6 antibody; HPS6 biogenesis of lysosomal organelles complex 2 subunit 3 antibody; HPS6_HUMAN antibody; MGC20522 antibody; Ru antibody; Ruby eye protein homolog antibody; Ruby-eye protein homolog antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Hermansky-Pudlak syndrome 6 protein (221-319AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    FITC
  • 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
  • 產品提供形式:
    Liquid
  • 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

  • 功能:
    May regulate the synthesis and function of lysosomes and of highly specialized organelles, such as melanosomes and platelet dense granules. Acts as cargo adapter for the dynein-dynactin motor complex to mediate the transport of lysosomes from the cell periphery to the perinuclear region. Facilitates retrograde lysosomal trafficking by linking the motor complex to lysosomes, and perinuclear positioning of lysosomes is crucial for the delivery of endocytic cargos to lysosomes, for lysosome maturation and functioning.
  • 基因功能參考文獻:
    1. Identification of a novel mutation in HPS6 in an individual with hemophilia B shows that, although quite rare, patients may be diagnosed with two independent inherited bleeding disorders. No evidence of lung disease was found in this adult patient with Hermansky-Pudlak syndrome subtype 6 PMID: 27641950
    2. the novel loss-of-function variant in the HPS6 subunit of biogenesis of lysosome-related organelles complex 2 is pathologic and leads to a reduced platelet dense granules and their release. The findings are compatible with an impaired platelet function and hence an enhanced bleeding risk. PMID: 27917594
    3. Biallelic, truncating mutations in HPS6 were identified by candidate Sanger sequencing and included a novel variant. PMID: 26823395
    4. we report novel HPS6 mutations as the first report of HPS6 mutations in the Japanese population. The clinical features in the two sisters suggest OA. Although the patients in this study showed no bleeding problem, we could establish a diagnosis of HPS-6 by WES. PMID: 27225848
    5. HPS6 interacts with dynactin p150Glued to mediate retrograde trafficking and maturation of lysosomes PMID: 25189619
    6. Mutation of the protein-trafficking gene Hps6 increased sensitivity of melanoma cells to cis-diaminedichloroplatinum II treatment. PMID: 22203954
    7. Molecular studies showed a variety of mutations in the single exon HPS6 gene, including frame shift, missense, and nonsense mutations as well as a approximately 20 kb deletion spanning the entire HPS6 genomic region. PMID: 19843503
    8. Component of BLOC-2. Results suggest a common biological basis underlying the pathogenesis of HPS-3, -5 and -6 disease. PMID: 15030569

    顯示更多

    收起更多

  • 相關疾病:
    Hermansky-Pudlak syndrome 6 (HPS6)
  • 亞細胞定位:
    Microsome membrane. Cytoplasm, cytosol. Early endosome membrane. Lysosome membrane.
  • 組織特異性:
    Ubiquitous.
  • 數據庫鏈接:

    HGNC: 18817

    OMIM: 607522

    KEGG: hsa:79803

    STRING: 9606.ENSP00000299238

    UniGene: Hs.125133



主站蜘蛛池模板: 国产成人国拍亚洲精品| 国产永久免费观看视频| 午夜福利麻豆国产精品| 久久国产精品日本波多野结衣| 欧美 日韩版国产在线播放| 一个人看的日本hd免费| 综合图区亚洲另类偷窥| 亚洲人成在线观看影院牛大爷| 护士被强女千到高潮视频 | av天堂永久资源网亚洲高清| 好吊射视频988gaocom| 巨大欧美黑人xxxxbbbb| 久久国产精品福利一区二区三区| 国产福利在线视频蜜芽tv| 欧美日产欧美日产国产精品| 成人婷婷网色偷偷亚洲男人的天堂| 欧美性受xxxx黑人xyx性爽| 国产成人亚洲高清一区| 日产a一a区二区www| 水蜜桃色314在线观看| 吃奶摸下的激烈视频| 做性久久久久久| 中文无码天天av天天爽| 欧美一区二区三区久久综合| 无码人妻精品丰满熟妇区| 亚洲男人的天堂在线aⅴ视频| 日本熟妇浓毛hdsex| 亚洲另类无码专区国内精品| 久久亚洲精品无码av宋| 久久久精品人妻无码专区不卡| 久久精品中文字幕一区二区三区| 中国农村妇女真实bbwbbwbbw| 日韩不卡1卡2卡三卡网站| 国产欠欠欠18一区二区| 久久www色情成人免费| 老湿机香蕉久久久久久| 亚洲日韩欧美国产高清αv| 免费人成在线观看网站品善网| 天天摸天天做天天添欧美| 精品国产天线2019| 无码av天天av天天爽|