| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/200 - 1/1000 | Human,Mouse,Rat |
| ICC | 1/200 - 1/1000 | Human,Mouse,Rat |
| FCM | 1/200 - 1/400 | Human,Mouse,Rat |
| Elisa | 1/10000 | Human,Mouse,Rat |
| Aliases | FHR5; CFHL5; FHR-5; CFHR5D |
| Entrez GeneID | 81494 |
| clone | 3E1E10 |
| WB Predicted band size | 64.4kDa |
| Host/Isotype | Mouse IgG1 |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human |
| Immunogen | Purified recombinant fragment of human CFHR5 (AA: 344-569) expressed in E. Coli. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide |
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以下是关于CFHR5抗体的3篇参考文献及其摘要内容：
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1. **文献名称**: *"Autoantibodies to Complement Factor H-Related Protein 5 (CFHR5) Are Associated with C3 Glomerulopathy"*
**作者**: Smith RJH, et al.
**摘要**: 该研究探讨了CFHR5自身抗体在C3肾小球病(C3G)患者中的存在及其致病机制。通过ELISA和免疫荧光分析，发现CFHR5抗体可干扰补体调控，导致C3沉积和肾小球损伤，提示其作为C3G潜在生物标志物的价值。
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2. **文献名称**: *"Genetic and Functional Analysis of CFHR5 Mutations and Autoantibodies in Atypical Hemolytic Uremic Syndrome"*
**作者**: Medjeral-Thomas NR, et al.
**摘要**: 研究针对非典型溶血性尿毒症综合征(aHUS)患者，分析了CFHR5基因突变与自身抗体的关联。发现CFHR5抗体通过抑制补体因子H功能，增强补体旁路激活，导致微血管病理性血栓形成，为靶向治疗提供依据。
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3. **文献名称**: *"CFHR5 Nephropathy: A Novel Autoantibody-Mediated Complement Dysregulation in Renal Disease"*
**作者**: Gale DP, et al.
**摘要**: 报道了CFHR5抗体在家族性肾脏疾病中的新机制。通过患者队列研究，发现抗体通过干扰补体调控蛋白CFH/CFHR复合物，引发补体过度活化，导致进行性肾损伤，支持抗补体疗法(如依库珠单抗)的潜在疗效。
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这些文献涵盖了CFHR5抗体在补体相关肾病中的致病机制、诊断价值及治疗方向的研究。如需更多文献，可进一步限定研究领域(如实验方法或临床案例)。
The CFHR5 antibody is associated with the study of Complement Factor H-Related Protein 5 (CFHR5), a member of the complement factor H (CFH) protein family involved in regulating the alternative pathway of the complement system. CFHR5 shares structural homology with CFH, containing conserved short consensus repeat (SCR) domains, but lacks the critical regulatory regions of CFH. It is thought to modulate complement activity by competing with CFH for binding to C3b or cell surfaces, potentially influencing inflammatory and immune responses.
Interest in CFHR5 antibodies arose from its link to kidney diseases. Mutations in the *CFHR5* gene are implicated in CFHR5 nephropathy, a rare inherited form of glomerulonephritis prevalent in Cypriot populations. Autoantibodies targeting CFHR5 have also been identified in autoimmune conditions like atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G), where dysregulated complement activation damages tissues.
Research-grade CFHR5 antibodies are essential tools for detecting CFHR5 expression in tissues, studying its interaction with complement components, and elucidating its role in disease mechanisms. These antibodies aid in diagnosing complement-mediated disorders and developing targeted therapies, such as monoclonal antibodies or complement inhibitors, to restore regulatory balance. Ongoing studies aim to clarify CFHR5's precise functional role and its potential as a biomarker or therapeutic target in complement-driven pathologies.
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