| WB | 1/500 - 1/2000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/100-1/1000 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/10000 | Human,Mouse,Rat |
| Aliases | PI; A1A; AAT; PI1; A1AT; SERPINA1 |
| Entrez GeneID | 5265 |
| clone | 2B12 |
| WB Predicted band size | 47kDa |
| 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,Mouse |
| Immunogen | Purified recombinant fragment of human AAT expressed in E. Coli. |
| Formulation | Ascitic fluid containing 0.03% sodium azide. |
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以下是关于AAT(α-1抗胰蛋白酶)抗体的3篇示例文献摘要，内容基于研究领域常见方向构建：
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1. **文献名称**：《Autoantibodies to Alpha-1 Antitrypsin in Systemic Lupus Erythematosus》
**作者**：Smith J, et al.
**摘要**：本研究检测了系统性红斑狼疮(SLE)患者血清中抗AAT自身抗体的水平，发现其与疾病活动度呈正相关，提示AAT抗体可能通过干扰蛋白酶-抗蛋白酶平衡参与SLE的病理进程。
2. **文献名称**：《Characterization of Anti-AAT Antibodies in Alpha-1 Antitrypsin Deficiency》
**作者**：Jones R, et al.
**摘要**：文章分析了AAT缺陷症患者中针对突变型AAT蛋白(如Z变异体)的自身抗体产生机制，发现抗体可通过促进肝细胞内AAT聚合物沉积加剧肝损伤，为靶向治疗提供新思路。
3. **文献名称**：《Monoclonal Antibodies for Detection of Alpha-1 Antitrypsin in Clinical Samples》
**作者**：Brown K, et al.
**摘要**：该研究开发了一种高特异性抗AAT单克隆抗体，优化了ELISA和免疫组化检测方法，显著提高了AAT缺乏症的诊断准确性，并验证了其在多种样本类型中的临床应用潜力。
4. **文献名称**：《Neutralizing Antibodies Targeting Protease Activity in AATD-related Emphysema》
**作者**：Wilson M, et al.
**摘要**：研究设计了一种中和性抗体，通过抑制中性粒细胞弹性蛋白酶的过度活性，减轻AAT缺乏导致的肺气肿病变，动物模型显示其可减少肺组织破坏并改善功能指标。
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注：以上文献为示例性内容，实际引用需查询真实数据库(如PubMed)。如需具体文献，建议通过关键词“Alpha-1 Antitrypsin antibody”在学术平台检索。
**Background of AAT Antibodies**
Alpha-1 antitrypsin (AAT), a serine protease inhibitor produced primarily in the liver, plays a critical role in protecting tissues, especially the lungs, from damage caused by neutrophil-derived enzymes like elastase. Genetic mutations in the *SERPINA1* gene can lead to AAT deficiency (AATD), a condition associated with early-onset emphysema, liver cirrhosis, and other disorders. AAT antibodies, specifically monoclonal or polyclonal immunoglobulins, are tools used to detect and quantify AAT levels in clinical or research settings.
In diagnostics, AAT antibodies are employed in techniques such as ELISA, immunonephelometry, or immunohistochemistry to identify AAT deficiency or abnormal protein accumulation in liver biopsies. They also aid in phenotyping genetic variants (e.g., Z or S alleles) that impact AAT functionality. Beyond diagnostics, these antibodies are pivotal in studying AAT’s role in inflammatory processes, autoimmune diseases, and its interaction with pathogens. Notably, autoantibodies against AAT have been observed in rare autoimmune conditions, potentially altering its protease-inhibiting capacity.
Therapeutic applications include monitoring AAT replacement therapy in AATD patients or evaluating experimental treatments like gene editing. Research continues to explore AAT antibodies' utility in understanding disease mechanisms and developing targeted therapies. Their specificity and adaptability make them indispensable in both clinical management and molecular studies of AAT-related pathologies.
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