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, Biotin conjugated

  • 中文名稱:
    HPS6兔多克隆抗體, Biotin偶聯(lián)
  • 貨號:
    CSB-PA803158LD01HU
  • 規(guī)格:
    ¥880
  • 其他:

產(chǎn)品詳情

  • 產(chǎn)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(yīng)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:
    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

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(guān)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(shù)據(jù)庫鏈接:

    HGNC: 18817

    OMIM: 607522

    KEGG: hsa:79803

    STRING: 9606.ENSP00000299238

    UniGene: Hs.125133



主站蜘蛛池模板: 99国内精品久久久久久久| 伊人久久大香线蕉影院| 亚洲精品无播放器在线播放| 国产亚洲精品美女久久久久| 天天看片视频免费观看| 性xxxxx欧美极品少妇| 热re99久久精品国产99热| 精品av无码国产一区二区| 国产亚洲日韩在线播放更多| 国产成人毛片无码视频软件| 亚洲老熟女av一区二区在线播放| 亚洲 成人 无码 在线观看| 国产jjzzjjzz视频全部免费| 免费无码av片在线观看网址| 少妇高潮大叫好爽| 亚洲精品国产一区二区贰佰信息网| 国产精品久久久久秋霞鲁丝| 最新国产精品久久精品| 国产精品日韩欧美一区二区三区| 朝鲜女人大白屁股ass| 黑人尾随强伦姧人妻爽翻天| 少妇的渴望hd高清在线播放| 国产卡一卡二卡三无线乱码新区| 97碰碰碰免费公开在线视频| 日本护士xxxxhd少妇| 国产肉体xxxx裸体784大胆| 午夜成人亚洲理伦片在线观看| 18禁黄网站禁片免费观看不卡| 在线亚洲专区高清中文字幕| 特级做a爰片毛片免费69| 性色a∨精品高清在线观看| 无码av在线一本无码| 日本乱偷人妻中文字幕| 日本少妇被黑人xxxxx| 黑人巨大videos精品| 亚洲 欧美 唯美 国产 伦 综合| 日韩va中文字幕无码电影| 精品亚洲欧美自拍| 亚洲图片综合图区20p| 女人被男人爽到呻吟的视频| 亚洲中文字幕在线无码一区二区|