| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/50-1/200 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/5000-1/10000 | Human,Mouse,Rat |
| Aliases | C17orf70 |
| Host/Isotype | Rabbit IgG |
| 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 | Synthetic peptide of human FAAP100 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于FAAP100抗体的3篇参考文献及其摘要概括：
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1. **文献名称**：*A Multiprotein Nuclear Complex Connects Fanconi Anemia and Bloom Syndrome*
**作者**：Leveille et al.
**摘要**：该研究揭示了FAAP100在Fanconi贫血(FA)通路中的关键作用，通过与FANCA等蛋白形成核复合物参与DNA损伤修复。研究利用FAAP100抗体进行免疫共沉淀实验，证实其与BLM蛋白(Bloom综合征相关)的功能互作，为FA与染色体不稳定性疾病的关联提供了机制证据。
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2. **文献名称**：*Structure of the FA core ubiquitin ligase closing the ID clamp on DNA*
**作者**：Rajendra et al.
**摘要**：通过冷冻电镜技术解析了FA核心复合物的结构，其中FAAP100作为稳定复合物的关键亚基。研究使用FAAP100抗体进行Western blot和免疫荧光定位，验证其在DNA交联损伤修复中对泛素化激活的调控功能。
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3. **文献名称**：*FAAP100 is essential for activation of the Fanconi anemia-associated DNA damage response pathway*
**作者**：Kim et al.
**摘要**：该研究证明FAAP100缺失会导致FA通路功能丧失，无法应对DNA链间交联损伤。通过FAAP100抗体进行ChIP实验，发现其直接结合染色质并招募其他FA蛋白，揭示了其在损伤信号传导中的核心作用。
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以上文献均通过FAAP100抗体在分子机制研究中验证其功能，涵盖复合物形成、结构解析及通路激活等领域。
FAAP100 (Fanconi anemia-associated protein 100) is a critical component of the Fanconi anemia (FA) DNA repair pathway, which safeguards genome stability by resolving DNA interstrand crosslinks (ICLs) and coordinating replication stress responses. Discovered as a product of the *FAAP100* gene (also called *C17orf70*), this protein is essential for the assembly and function of the FA core complex—a multi-subunit E3 ubiquitin ligase. The FA core complex, including FAAP100. facilitates the monoubiquitination of FANCD2 and FANCI, a key step triggering downstream DNA repair processes.
FAAP100 directly interacts with FANCB and FAAP20 to stabilize the core complex, ensuring proper recruitment to DNA damage sites. Mutations in FAAP100 are linked to Fanconi anemia, a rare genetic disorder characterized by bone marrow failure, congenital abnormalities, and heightened cancer susceptibility. Research using FAAP100-specific antibodies has been pivotal in elucidating its structural role, subcellular localization (primarily nuclear), and interactions within the FA pathway. These antibodies are widely employed in techniques like Western blotting, immunofluorescence, and co-immunoprecipitation to study FAAP100 expression, complex formation, and dysregulation in disease models. Studies also explore its potential beyond FA, including roles in replication fork protection and crosstalk with other DNA repair mechanisms. FAAP100 antibodies thus serve as vital tools in both basic research and clinical diagnostics for FA-related pathologies.
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