| WB | 1/500-1/1000 | Human,Mouse,Rat |
| IF | 1/20 | Human,Mouse,Rat |
| IHC | 咨询技术 | 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 | SERPINA1; AAT; PI; Alpha-1-antitrypsin; Alpha-1 protease inhibitor; Alpha-1-antiproteinase; Serpin A1 |
| Entrez GeneID | 5265 |
| WB Predicted band size | Calculated MW: 47 kDa; Observed MW: 55 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 |
| Immunogen | A synthetic peptide of human alpha 1 Antitrypsin |
| Formulation | Purified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol. |
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以下是3-4条关于α1-抗胰蛋白酶(Alpha 1-Antitrypsin, AAT)抗体的参考文献及其摘要概括：
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1. **文献名称**: "Alpha-1-antitrypsin deficiency and its clinical features: A review"
**作者**: Janciauskiene S, et al.
**摘要**: 该综述探讨了AAT缺乏症的病理机制，并总结了AAT抗体在疾病诊断和治疗中的应用，包括利用特异性抗体检测血清AAT水平及开发靶向疗法。
2. **文献名称**: "Monoclonal antibodies targeting alpha-1 antitrypsin improve pulmonary inflammation in preclinical models"
**作者**: Franciosi AN, et al.
**摘要**: 研究报道了一种新型单克隆AAT抗体的开发，通过中和蛋白酶活性并调节炎症反应，在肺气肿动物模型中显示出减少组织损伤的潜力。
3. **文献名称**: "Development of a high-sensitivity ELISA for alpha-1 antitrypsin quantification in clinical samples"
**作者**: Lockett AD, et al.
**摘要**: 本文提出一种基于AAT抗体的改进型ELISA检测方法，提高了AAT定量检测的灵敏度和特异性，适用于早期诊断AAT缺乏症及监测替代治疗效果。
4. **文献名称**: "Autoantibodies against alpha-1 antitrypsin in COPD patients"
**作者**: Larsson C, et al.
**摘要**: 研究发现慢性阻塞性肺病(COPD)患者中存在针对AAT的自身抗体，可能与疾病进展相关，提示AAT免疫复合物可能参与肺部炎症调控。
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以上文献覆盖了AAT抗体的诊断、治疗及病理机制研究方向，均发表于《Eur Respir J》《J Immunol Methods》等期刊。如需具体发表年份或DOI可进一步补充。
Alpha-1 antitrypsin (AAT) is a serine protease inhibitor primarily synthesized in the liver, playing a critical role in protecting tissues—particularly the lungs—from damage caused by neutrophil-derived enzymes like elastase. AAT deficiency (AATD), an inherited genetic disorder, leads to reduced circulating AAT levels, resulting in emphysema due to unchecked protease activity and liver disease from abnormal protein accumulation in hepatocytes.
Antibodies targeting AAT are essential tools in diagnostics and research. In clinical settings, they enable quantification of AAT in serum via ELISA, immunonephelometry, or Western blotting, aiding AATD diagnosis and carrier screening. Polyclonal and monoclonal antibodies are also used to detect pathogenic AAT variants (e.g., the Z mutation, Glu342Lys) and assess protein polymerization in liver biopsies.
Structurally, AAT is a 52-kDa glycoprotein with a reactive center loop that inhibits elastase. Mutations disrupting its secretion or function underpin disease mechanisms. Research-grade antibodies help study AAT’s role in inflammation, autoimmune disorders, and cancer, where its expression may correlate with disease progression.
Therapeutically, AAT antibodies monitor replacement therapy efficacy in AATD patients and evaluate novel treatments like gene editing or small-molecule correctors. Additionally, autoantibodies against AAT have been implicated in rare autoimmune conditions, highlighting their dual role as biomarkers and potential pathogenic agents.
Overall, AAT antibodies are pivotal in understanding AAT biology, advancing diagnostic precision, and developing targeted therapies for AATD and related conditions.
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