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HPS3 Antibody

  • 货号：
    CSB-PA010712GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q969F9
  • 基因名：
    HPS3
  • 别名：
    HPS3 antibody; Hermansky-Pudlak syndrome 3 protein antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human HPS3
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 功能：
    Involved in early stages of melanosome biogenesis and maturation.
  • 基因功能参考文献：
    1. HPS3 mutation is associated with high hypopigmentation in Hermansky-Pudlak syndrome. PMID: 27593200
    2. Description of mutations in HPS genes that cause Hermansky-Pudlak syndrome (review) PMID: 12125811
    3. HPS4 but not HPS3 associates with HPS1 in a complex, which we term biogenesis of lysosome-related organelles complex 3 (BLOC-3) PMID: 12847290
    4. A specific subset of melanocyte proteins are aberrantly trafficked throughout the HPS-3 melanocyte and may be responsible for the reduction in melanin synthesis. PMID: 15632015
    5. HPS3 associates with clathrin, predominantly on small clathrin-containing vesicles in the perinuclear region. This association most likely occurs directly via a functional clathrin-binding domain in HPS3. PMID: 16159387
    6. mutational analysis in Ashkenazi Jews with Hermansky-Pudlak syndrome 3 PMID: 11590544

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  • 相关疾病：
    Hermansky-Pudlak syndrome 3 (HPS3)
  • 亚细胞定位：
    Cytoplasm. Cytoplasm, cytosol.
  • 组织特异性：
    Widely expressed. Higher levels of expression are observed in kidney, liver and placenta.
  • 数据库链接：

    HGNC: 15597

    OMIM: 606118

    KEGG: hsa:84343

    STRING: 9606.ENSP00000296051

    UniGene: Hs.558314