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  4. HPS6 Antibody

HPS6 Antibody

  • 中文名稱：
    HPS6兔多克隆抗體
  • 貨號：
    CSB-PA803158LA01HU
  • 規格：
    ￥440
  • 圖片：
    • IHC image of CSB-PA803158LA01HU diluted at 1:400 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • IHC image of CSB-PA803158LA01HU diluted at 1:400 and staining in paraffin-embedded human adrenal gland tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • Immunofluorescence staining of Hela cells with CSB-PA803158LA01HU at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 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：
    Non-conjugated

    本頁面中的產品，HPS6 Antibody (CSB-PA803158LA01HU)，的標記方式是Non-conjugated。對于HPS6 Antibody，我們還提供其他標記。見下表：

    可提供標記
    標記方式 貨號 產品名稱 應用
    HRP CSB-PA803158LB01HU HPS6 Antibody, HRP conjugated ELISA
    FITC CSB-PA803158LC01HU HPS6 Antibody, FITC conjugated
    Biotin CSB-PA803158LD01HU HPS6 Antibody, Biotin conjugated ELISA
  • 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, IHC, IF
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:200-1:500
    IF 1:50-1:200
  • 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：
    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

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  • 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



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