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Mouse Monoclonal PRF1 Antibody

  • 中文名: PRF1抗体
  • 别    名: P1; PFP; HPLH2
货号: IPD32165
Price: ¥1280
数量:
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验证与应用

应用及物种
WB 咨询技术 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 咨询技术 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 1/200 - 1/400 Human,Mouse,Rat
Elisa 1/10000 Human,Mouse,Rat

产品详情

AliasesP1; PFP; HPLH2
Entrez GeneID5551
clone6F8C2
WB Predicted band size61.3kDa
Host/IsotypeMouse IgG1
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman
ImmunogenPurified recombinant fragment of human PRF1 (AA: extra 148-372) expressed in E. Coli.
FormulationPurified antibody in PBS with 0.05% sodium azide

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参考文献

以下是关于PRF1抗体的参考文献及摘要概括:

1. **"Perforin gene defects in familial hemophagocytic lymphohistiocytosis"**

- **作者**: Stepp, S.E., et al.

- **摘要**: 该研究分析了PRF1基因突变导致的家族性噬血细胞性淋巴组织细胞增生症(FHL),发现患者中穿孔素表达缺失。文中利用PRF1特异性抗体通过免疫组化和流式细胞术检测淋巴细胞,证实了抗体在诊断遗传性免疫缺陷中的关键作用。

2. **"The structural basis of perforin assembly and function"**

- **作者**: Voskoboinik, I., et al.

- **摘要**: 通过X射线晶体学和抗体标记技术,揭示了穿孔素蛋白的分子结构及其在靶细胞膜孔形成中的作用。研究强调了PRF1抗体在解析其功能机制及疾病相关突变中的实验应用。

3. **"Perforin deficiency in human patients modulates NK cell-mediated cytotoxicity"**

- **作者**: Côte, M., et al.

- **摘要**: 探讨PRF1缺陷患者的自然杀伤细胞(NK)功能异常,采用抗PRF1抗体进行Western blot和免疫荧光分析,证明穿孔素缺失直接导致细胞毒性功能障碍,为免疫治疗提供依据。

4. **"Diagnostic relevance of PRF1 antibody staining in hemophagocytic syndromes"**

- **作者**: Sieni, E., et al.

- **摘要**: 评估PRF1抗体在临床诊断中的价值,通过流式细胞术和免疫组化检测患者样本,发现抗体可有效区分FHL与其他类似疾病,优化了快速诊断流程。

这些文献涵盖了PRF1抗体在基础研究、结构解析、疾病机制及临床诊断中的应用。

背景信息

The PRF1 antibody targets perforin-1. a cytotoxic effector protein encoded by the PRF1 gene. Perforin-1 is predominantly expressed in natural killer (NK) cells and cytotoxic T lymphocytes (CTLs), where it plays a critical role in immune defense by forming pores in target cell membranes. This pore-forming activity facilitates the entry of pro-apoptotic granzymes, triggering programmed cell death in virus-infected or malignant cells. PRF1 antibodies are essential tools for studying immune cell function, particularly in contexts like cancer immunology, viral infections, and autoimmune disorders. They are widely used in techniques such as Western blotting, immunohistochemistry (IHC), and flow cytometry to detect perforin-1 expression levels or localization in tissues and immune cells. Clinically, PRF1 mutations are linked to familial hemophagocytic lymphohistiocytosis (FHL type 2), a severe immunodeficiency disorder characterized by defective cytotoxic granule-mediated apoptosis. PRF1 antibodies aid in diagnosing such conditions by identifying protein deficiencies or abnormal expression patterns. Additionally, research applications include investigating immune evasion mechanisms in tumors and evaluating therapeutic interventions aimed at enhancing cytotoxic immune responses. Validation of PRF1 antibody specificity is crucial, as cross-reactivity with unrelated proteins may lead to inaccurate interpretations in both research and diagnostic settings.

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