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, HRP conjugated

  • 中文名稱:
    HPS6兔多克隆抗體, HRP偶聯
  • 貨號:
    CSB-PA803158LB01HU
  • 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:
    HRP
  • 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
  • 應用范圍:
    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: 在线亚洲综合欧美网站首页| 国内揄拍高清国内精品对白 | 成人福利国产精品视频| 人妻无码精品久久亚瑟影视| 久久午夜无码鲁丝片| 欧美裸体xxxx极品少妇| 野花社区在线www日本| 亚洲欧洲日产韩国无码| 337p日本欧洲亚洲大胆精筑 | 野花在线无码视频在线播放| 精品无码久久久久国产app| 国产精品久久久久久福利| 狠狠综合久久av一区二区| 波多野结衣av在线无码中文18| 精品日产一卡二卡| 十八禁无码免费网站| 国产综合无码一区二区色蜜蜜| 国产精品毛片av在线看| 老司机免费的精品视频| 顶级欧美做受xxx000| 轻点好疼好大好爽视频| av在线亚洲男人的天堂| 欧美日韩精品suv| 亚洲色欲色欲www在线看| 国产人妻精品久久久久野外| 嫖妓丰满肥熟妇在线精品| 久久久一本精品久久精品六六| 一本一道久久a久久精品综合| 成 人影片 免费观看| 亚洲综合日韩久久成人av| 三级全黄的视频在线观看| 深夜福利在线观看视频| 97无码免费人妻超级碰碰夜夜| 乱无码伦视频在线观看| 少妇群交换bd高清国语版| 亚洲欧美日韩综合一区二区 | 天干天干夜啦天干天干国产| 亚洲图片小说激情综合| 色135综合网| 国产高清午夜人成在线观看| 欧美自拍亚洲综合丝袜|