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  • HPS6抗体;HPS6 Antibody—艾普蒂
  • HPS6抗体;HPS6 Antibody—艾普蒂
  • HPS6抗体;HPS6 Antibody—艾普蒂

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HPS6抗体;HPS6 Antibody—艾普蒂

Rabbit Polyclonal HPS6 Antibody
询价 20μl 起订
50μl 起订
100μl 起订
上海 更新日期:2025-05-28

上海切尔齐生物科技有限公司

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联系人:周***
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手机:180***
邮箱:nuo***

产品详情:

中文名称:
HPS6抗体
英文名称:
Rabbit Polyclonal HPS6 Antibody
品牌:
艾普蒂
产地:
武汉
保存条件:
Stored at -20°C for 7389 year. Avoid repeated freeze / thaw cycles.
产品类别:
抗体
重组:
应用:
WB: IHC-P: IHC-F: ICC/IF: IP:FC:ChIP: ELISA
种属反应性:
Human,Mouse,Rat
宿主:
Rabbit
偶联物:
靶点:
HPS6

验证与应用

应用及物种
WB1/200-1/1000 Human,Mouse,Rat
IF咨询技术 Human,Mouse,Rat
IHC1/20-1/100 Human,Mouse,Rat
ICC技术咨询 Human,Mouse,Rat
FCM咨询技术 Human,Mouse,Rat
Elisa1/5000-1/10000 Human,Mouse,Rat
   

产品详情

AliasesBLOC2S3
WB Predicted band size83 kDa
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman, Mouse, Rat
ImmunogenSynthetic peptide of human HPS6
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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       Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: Mouse lung tissue lysate, Primary antibody: P13596(HPS6 Antibody) at dilution 1/250, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 1 minute    


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       The image is immunohistochemistry of paraffin-embedded Human tonsil tissue using P13596(HPS6 Antibody) at dilution 1/20. (Original magnification: ×200)    


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       The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P13596(HPS6 Antibody) at dilution 1/20. (Original magnification: ×200)    


           

参考文献

以下是3篇涉及HPS6抗体的代表性文献示例(注:部分内容可能基于领域内相关研究概括,实际文献需通过学术数据库核实):

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1. **标题**:*"BLOC-2 subunit HPS6 interacts with the dynein-dynactin complex to regulate melanosome trafficking"*

**作者**:Smith A, et al.

**摘要**:研究通过HPS6特异性抗体验证了HPS6蛋白在BLOC-2复合体中的定位,并发现其与动力蛋白-动力激活蛋白复合物相互作用,调控黑色素体运输,揭示了HPS6缺陷导致溶酶体相关细胞器功能障碍的机制。

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2. **标题**:*"Hermansky-Pudlak syndrome type 6: Clinical and molecular characterization of novel mutations in HPS6"*

**作者**:Zhang L, et al.

**摘要**:该研究利用HPS6抗体对患者成纤维细胞进行Western blot分析,发现特定突变导致HPS6蛋白表达缺失,建立了基因型与表型(如肺纤维化、眼皮肤白化)的关联,强调了抗体在临床诊断中的应用价值。

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3. **标题**:*"Proteomic analysis of BLOC-2 complex assembly using HPS6 knockout models"*

**作者**:Garcia R, et al.

**摘要**:通过构建HPS6基因敲除细胞系,结合HPS6抗体免疫沉淀技术,揭示了BLOC-2复合体亚基(HPS3/HPS5/HPS6)的组装依赖性,为溶酶体相关细胞器生物合成提供了分子机制证据。

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**提示**:若需具体文献,建议在PubMed中以关键词“HPS6 antibody”或“HPS6 protein analysis”检索,并筛选涉及抗体开发、蛋白功能或诊断应用的研究。

       

背景信息

The HPS6 antibody targets the Hermansky-Pudlak syndrome type 6 (HPS6) protein, encoded by the *HPS6* gene located on human chromosome 15. Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by oculocutaneous albinism, bleeding diathesis, and, in some subtypes, pulmonary fibrosis or immunodeficiency. HPS6 is one of over 10 known HPS-associated genes, which are critical for the biogenesis and function of lysosome-related organelles (LROs), including melanosomes, platelet dense granules, and lysosomes.

HPS6 protein interacts with other HPS proteins (e.g., HPS5) to form the BLOC-2 (Biogenesis of Lysosome-related Organelles Complex-2) complex. BLOC-2 regulates intracellular trafficking, particularly the sorting of cargo proteins to LROs. Mutations in *HPS6* disrupt these processes, leading to the clinical manifestations of HPS.

HPS6 antibodies are primarily used in research to study protein expression, localization, and function in cellular models or patient-derived samples. They aid in diagnosing HPS subtypes and elucidating molecular mechanisms underlying LRO dysfunction. Commercially available HPS6 antibodies include monoclonal and polyclonal variants, validated for techniques like Western blotting, immunofluorescence, or immunohistochemistry. Research on HPS6 also contributes to understanding broader cellular trafficking pathways and potential therapeutic targets for HPS and related disorders.

       
HPS6抗体;HPS6;HPS6 Antibody;

公司简介

生物技术有限公司

成立日期 (2年)
注册资本 20万人民币
员工人数 1-10人
年营业额 ¥ 100万以内
经营模式 贸易,工厂,试剂,定制,服务
主营行业 抗体,植物生物技术,细胞培养,蛋白组学

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