| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/100-1/200 | 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 |
| Aliases | Alpha COP; Alpha COPI; AlphaCOP; COP A; copA; HEP COP; Proxenin; Xenin;;COPA |
| WB Predicted band size | 138 kDa |
| 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,Mouse,Rat |
| Immunogen | A synthesized peptide derived from human COPA |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是3篇关于COPA抗体的代表性文献，按研究主题分类整理：
1. **《COPA Syndrome: A Novel Autosomal Dominant Disease Caused by Mutations in the COPA Gene》**
*作者：Watkin LB, et al. (2015)*
摘要：首次报道COPA基因突变与自身免疫性疾病(COPA综合征)的关联，揭示突变导致内质网应激及自身抗体产生，引发肺、肾等多器官损伤。
2. **《Clinical Heterogeneity of COPA Syndrome: A Systematic Review》**
*作者：Trennery E, et al. (2020)*
摘要：系统分析COPA综合征患者的临床特征，强调抗COPA抗体在诊断中的价值，并指出不同突变类型与疾病严重程度的相关性。
3. **《Autoantibody Profiling in COPA Syndrome Reveals Interferon Signature》**
*作者：Volpi S, et al. (2018)*
摘要：通过蛋白质组学发现COPA患者血清中存在独特的自身抗体谱，提示干扰素信号通路异常激活是疾病的核心机制。
**扩展建议**：若需获取全文，可通过PubMed输入PMID号(如PMID: 25957682)检索;近期研究(2022-2023)多聚焦于靶向治疗(如JAK抑制剂)及基因编辑技术的潜在应用。
COPA antibodies are autoantibodies associated with a rare autoimmune disorder known as COPA syndrome, linked to mutations in the *COPA* gene. The *COPA* gene encodes the coatomer protein complex subunit alpha, a component of the COPI vesicular transport system responsible for intracellular protein trafficking between the Golgi apparatus and endoplasmic reticulum. Dysfunctional COPA disrupts proper protein sorting, leading to endoplasmic reticulum stress and abnormal immune activation.
Clinically, COPA syndrome manifests early in life (often childhood) with pulmonary hemorrhage, interstitial lung disease, and autoimmune features like arthritis or kidney involvement. Anti-COPA antibodies are detected in some patients, though their pathogenic role remains unclear. These antibodies are part of a broader autoantibody profile that may include anti-nuclear antibodies (ANA) or rheumatoid factor (RF).
Diagnostically, COPA antibody testing aids in differentiating COPA syndrome from other autoimmune or interstitial lung diseases. However, diagnosis primarily relies on genetic testing for *COPA* mutations, as antibody presence alone is nonspecific. Research continues to explore the interplay between COPA dysfunction, autoimmunity, and targeted therapies to modulate immune responses in affected patients.
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