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

HPS1 Antibody

  • 中文名稱:
    HPS1兔多克隆抗體
  • 貨號:
    CSB-PA010711GA01HU
  • 規格:
    ¥3,900
  • 其他:

產品詳情

  • Uniprot No.:
  • 基因名:
    HPS1
  • 別名:
    Hermansky Pudlak syndrome 1 protein antibody; Hermansky Pudlak syndrome gene antibody; Hermansky Pudlak syndrome type 1 antibody; Hermansky-Pudlak syndrome 1 antibody; Hermansky-Pudlak syndrome 1 protein antibody; HPS antibody; HPS1 antibody; HPS1_HUMAN antibody; MGC5277 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human,Mouse,Rat
  • 免疫原:
    Human HPS1
  • 免疫原種屬:
    Homo sapiens (Human)
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen Affinity purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA,WB
  • 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:
    Component of the BLOC-3 complex, a complex that acts as a guanine exchange factor (GEF) for RAB32 and RAB38, promotes the exchange of GDP to GTP, converting them from an inactive GDP-bound form into an active GTP-bound form. The BLOC-3 complex plays an important role in the control of melanin production and melanosome biogenesis and promotes the membrane localization of RAB32 and RAB38.
  • 基因功能參考文獻:
    1. HPS1 mutation is associated with high hypopigmentation in Hermansky-Pudlak syndrome. PMID: 27593200
    2. BLOC-3 is a Rab32 and Rab38 guanine nucleotide exchange factor, with a specific function in the biogenesis of lysosome-related organelles. Silencing of the BLOC-3 subunits Hps1 and Hps4 results in the mislocalization of Rab32 and Rab38. PMID: 23084991
    3. Seven mutations (six previously unreported) were described in the HPS1, HPS4, and HPS5 genes among Hermansky-Pudlak Syndrome patients of Mexican, Uruguayan, Honduran, Cuban, Venezuelan, and Salvadoran ancestries. PMID: 21833017
    4. a previously unreported missense mutation (G313S) at the 3' splice junction of exon 10 of Hermansky-Pudlak syndrome 1 protein resulted in activation of a cryptic intronic splice site causing an aberrantly spliced HPS1 mRNA PMID: 20662851
    5. Three different mutations in the HPS1 gene were found in the two families. PMID: 20514622
    6. Data show that recombinant HPS1-HPS4 produced in insect cells can be efficiently isolated as a 1:1 heterodimer, and might function as a Rab9 effector in the biogenesis of lysosome-related organelles. PMID: 20048159
    7. Description of mutations in HPS genes that cause Hermansky-Pudlak syndrome (review) PMID: 12125811
    8. Four novel mutations were discovered and the diagnosis of HPS-1, available only on molecular grounds, has important prognostic and treatment implications. PMID: 12442288
    9. identification as a component of two complexes, BLOC-3 and BLOC-4, involved in the biogenesis of lysosome-related organelles PMID: 12663659
    10. observations demonstrate that the Hermansky-Pudlak syndrome 1(HPS1) and HPS4 proteins are components of a cytosolic complex that is involved in the biogenesis of lysosomal-related organelles PMID: 12756248
    11. Biogenesis of lysosome-related organelles complex 3 (BLOC-3): a complex containing the Hermansky-Pudlak syndrome (HPS) proteins HPS1 and HPS4. PMID: 12847290
    12. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 1. PMID: 16020891
    13. The first case report of a Chinese Hermansky-Pudlak syndrome patient with a novel mutation on HPS1 gene. PMID: 19665357

    顯示更多

    收起更多

  • 相關疾病:
    Hermansky-Pudlak syndrome 1 (HPS1)
  • 組織特異性:
    Ubiquitous.
  • 數據庫鏈接:

    HGNC: 5163

    OMIM: 203300

    KEGG: hsa:3257

    STRING: 9606.ENSP00000326649

    UniGene: Hs.404568



主站蜘蛛池模板: 蜜芽久久人人超碰爱香蕉| 精品无码久久久久国产| 人妻去按摩店被黑人按中出| 久久综合久久美利坚合众国| 成人国产精品无码网站| 亚洲精品国偷自产在线99人热 | 闺蜜张开腿让我爽了一夜| 国产乱xxxxx97国语对白| 日本高清视频网站www| 涩里番网污站| 我的性奴的肉玩具1一17心奴| 欧美性xxxxx极品少妇直播| а√天堂www在线天堂小说| 成人亚洲a片v一区二区三区蜜臀| 国产成人啪精品视频免费视频| 久久久无码精品亚洲日韩啪啪网站| 成人免费无码av| 高清国产亚洲精品自在久久| 综合无码成人aⅴ视频在线观看| 日本大肚子孕妇交xxx| 欧美又粗又大xxxxbbbb疯狂| 精品人体无码一区二区三区| 伊人无码一区二区三区| 国产亚洲精品欧洲在线观看| 四虎免费大片aⅴ入口| 亚洲国产精彩中文乱码av | 色综合亚洲一区二区小说性色aⅴ| 国产精品无码一区二区三区免费| 欧美乱妇狂野欧美在线视频| 一区二区视频传媒有限公司| 717影院理论午夜伦八戒| 精品深夜av无码一区二区老年| 成人免费视频在线观看| 国产精品无码久久综合网| 人妻精品久久久久中文字幕69| 欧美性生 活18~19| 狠狠色噜噜狠狠狠狠色综合久av | 在教室伦流澡到高潮hgl视频| 娇妻玩4p被三个男人伺候电影| 成 人色 网 站 欧美大片在线观看| 欧美人妻久久精品|