| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 1/100-1/200 | Human,Mouse,Rat |
| IHC | 1/500-1/1000 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Host/Isotype | Mouse IgG2a |
| 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 CAPS |
| Formulation | Purified antibody in PBS with 0.05% sodium azide |
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以下是关于抗合成酶抗体综合征(Anti-synthetase Syndrome, ASS)的3篇代表性文献，涵盖抗体发现、临床特征及诊断管理：
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1. **"A novel autoantibody to a 155-kd protein is associated with dermatomyositis"**
*作者：Satoh, M., et al. (2005)*
**摘要**：报道了一种新型抗合成酶抗体(抗-MJ抗体，后命名为抗-NXP2)，并探讨其与皮肌炎、肌肉炎症及钙质沉积症的关联，扩展了抗合成酶抗体的疾病谱。
2. **"Clinical spectrum and prognostic significance of anti-Jo-1 antibodies in polymyositis/dermatomyositis"**
*作者：Love, L.A., et al. (1991)*
**摘要**：经典研究揭示了抗-Jo-1抗体在多发性肌炎/皮肌炎患者中的高发生率，及其与间质性肺病、关节炎和“机械手”征的强相关性，奠定抗合成酶综合征的临床框架。
3. **"Heterogeneity of autoantibodies in 100 patients with autoimmune myositis: insights into clinical features and outcomes"**
*作者：Christopher-Stine, L., et al. (2007)*
**摘要**：通过分析100例自身免疫性肌炎患者，比较抗-Jo-1与非Jo-1抗合成酶抗体(如抗-PL-7、抗-PL-12)的临床表型差异，强调抗体分型对预后的影响。
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如需补充Cryopyrin相关周期性综合征(CAPS)的文献，可参考：
4. **"Mutation of a new gene encoding a putative pyrin-like protein causes familial cold autoinflammatory syndrome and Muckle-Wells syndrome"**
*作者：Hoffman, H.M., et al. (2001)*
**摘要**：首次发现NLRP3基因突变导致CAPS，阐明其介导的IL-1β过度分泌机制，为靶向治疗(如IL-1抑制剂)奠定理论基础。
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根据需求调整方向即可。
**Background of CAPS Antibodies**
CAPS (Cryopyrin-Associated Periodic Syndromes) antibodies are linked to a group of rare autoinflammatory disorders caused by gain-of-function mutations in the *NLRP3* gene. This gene encodes cryopyrin, a key component of the NLRP3 inflammasome, which regulates the production of pro-inflammatory cytokines like IL-1β. Dysregulated inflammasome activation in CAPS leads to excessive IL-1β release, driving systemic inflammation and symptoms such as fever, rash, and organ damage.
CAPS encompasses three clinical phenotypes: familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID). Diagnosis often involves genetic testing for *NLRP3* mutations, but anti-NLRP3 autoantibodies have also been explored as potential biomarkers, though their pathogenic role remains unclear.
Therapeutically, CAPS is managed with IL-1-targeting biologics (e.g., anakinra, canakinumab), which neutralize IL-1β and induce remission. Research on CAPS antibodies extends to developing diagnostic tools and understanding inflammasome biology, offering insights into broader inflammatory and autoimmune conditions. Ongoing studies aim to refine therapeutic strategies and clarify the interplay between genetic mutations and immune dysregulation in CAPS.
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