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HPS1 Antibody

  • 中文名稱：
    HPS1兔多克隆抗體
  • 貨號：
    CSB-PA856438ESR1HU
  • 規格：
    ￥440
  • 圖片：
    • Immunohistochemistry of paraffin-embedded human small intestine tissue using CSB-PA856438ESR1HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human thymus tissue using CSB-PA856438ESR1HU at dilution of 1:100
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) HPS1 Polyclonal antibody
  • 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
  • 免疫原：
    Recombinant Human Hermansky-Pudlak syndrome 1 protein (1-324AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 克隆類型：
    Polyclonal
  • 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 0.02% sodium azide, 50% glycerol, pH7.3.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • 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

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  • 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



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