AAT Antibody (8.F.14): sc-69986

AAT Antibody (8.F.14) is a mouse monoclonal IgG1 antibody that detects α1-antitrypsin (AAT) in mouse, rat, and human samples through applications such as western blotting (WB), immunoprecipitation (IP), and enzyme-linked immunosorbent assay (ELISA). AAT is a crucial hepatic acute phase protein composed of 394 amino acids, primarily synthesized in the liver, where AAT plays a vital role in protecting lung tissue from damage caused by Neutrophil Elastase, a serine protease implicated in the development of pulmonary emphysema. AAT′s location in the liver is significant because this allows for rapid response to inflammatory stimuli, ensuring AAT is readily available to inhibit proteolytic enzymes that can lead to tissue destruction. AAT is a highly polymorphic glycosylated serum protein, exhibiting distinct isoelectric-focusing patterns for various genetic variants, which can influence functional capacity and therapeutic potential. The gene encoding AAT is located on human chromosome 14, adjacent to another serpin gene that encodes corticosteroid-binding globulin, highlighting the interconnectedness of these proteins in regulating inflammatory responses. Oxidation of the methionine 358 residue in AAT′s active site significantly impairs AAT′s ability to inhibit elastase, underscoring the importance of post-translational modifications in maintaining protective function. AAT deficiency is associated with a markedly increased risk of developing early-onset pulmonary emphysema, making AAT study and interactions critical for understanding and potentially mitigating lung diseases.

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AAT Antibody (8.F.14) References:

1. The selective advantage of alpha1-antitrypsin deficiency.  |  Lomas, DA. 2006. Am J Respir Crit Care Med. 173: 1072-7. PMID: 16439713
2. Alpha-1-antitrypsin (AAT) anomalies are associated with lung disease due to rapidly growing mycobacteria and AAT inhibits Mycobacterium abscessus infection of macrophages.  |  Chan, ED., et al. 2007. Scand J Infect Dis. 39: 690-6. PMID: 17654345
3. Proteostasis strategies for restoring alpha1-antitrypsin deficiency.  |  Bouchecareilh, M., et al. 2010. Proc Am Thorac Soc. 7: 415-22. PMID: 21030523
4. Anti-inflammatory and immunomodulatory properties of α1-antitrypsin without inhibition of elastase.  |  Jonigk, D., et al. 2013. Proc Natl Acad Sci U S A. 110: 15007-12. PMID: 23975926
5. Distinguishing alpha1-antitrypsin deficiency from asthma.  |  Siri, D., et al. 2013. Ann Allergy Asthma Immunol. 111: 458-64. PMID: 24267358
6. Off-target rewards of augmentation therapy with α1-antitrypsin.  |  Jenne, DE. 2014. Am J Respir Crit Care Med. 190: 1203-4. PMID: 25436777
7. α1-antitrypsin promotes lung adenocarcinoma metastasis through upregulating fibronectin expression.  |  Li, Y., et al. 2017. Int J Oncol. 50: 1955-1964. PMID: 28440399
8. In Vivo Genome Editing Partially Restores Alpha1-Antitrypsin in a Murine Model of AAT Deficiency.  |  Song, CQ., et al. 2018. Hum Gene Ther. 29: 853-860. PMID: 29597895
9. α1-Antitrypsin Causing CD8 Downregulation during Thymocyte Differentiation.  |  Bristow, CL. and Winston, R. 2021. Trends Immunol. 42: 177-178. PMID: 33546997
10. alpha 1-Antitrypsin (AAT) deficiency and ANCA-positive systemic vasculitis: genetic and clinical implications.  |  Callea, F., et al. 1997. Eur J Clin Invest. 27: 696-702. PMID: 9279535

The preferred AAT antibody is AAT Antibody (B9): sc-59438.