ReviewLung
Alpha-1-antitrypsin deficiency: current concepts.
Review in Lung. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
15 citing papers in PubMed.
- Polymeric alpha-1 antitrypsin perturbs Paneth cell proteostasis and exacerbates intestinal inflammation.Signal transduction and targeted therapy · 2026Article
- Chaperone-mediated autophagy degrades SERPINA1Autophagy · 2025Article
- Strategy for reproductive and developmental toxicity (DART) studies for marketing applications in pharmaceutical development.Current research in toxicology · 2025Article
- Comparison of alpha 1- antitrypsin activity and phenotype in type 1 diabetic patients to healthy individuals.Journal of family medicine and primary care · 2022Article
- Review
- Alpha-1-antitrypsin therapy ameliorates acute colitis and chronic murine ileitis.Inflammatory bowel diseases · 2013Article
- Proteomic analyses of human plasma: Venus versus Mars.Transfusion · 2012Article
- Pilot analysis of the plasma metabolite profiles associated with emphysematous Chronic Obstructive Pulmonary Disease phenotype.Biochemical and biophysical research communications · 2011Article
- Acquired alpha 1-antitrypsin deficiency in tropical pulmonary eosinophilia.The Indian journal of medical research · 2011Article
- Role of LTB₄ in the pathogenesis of elastase-induced murine pulmonary emphysema.American journal of physiology. Lung cellular and molecular physiology · 2010Article
- Proteostasis strategies for restoring alpha1-antitrypsin deficiency.Proceedings of the American Thoracic Society · 2010Review
- Transcription factor PrtT controls expression of multiple secreted proteases in the human pathogenic mold Aspergillus fumigatus.Infection and immunity · 2009Article
- Article
- Alveolar epithelial and endothelial cell apoptosis in emphysema: what we know and what we need to know.International journal of chronic obstructive pulmonary disease · 2009Review
- Chitosan-genipin nanohydrogel as a vehicle for sustained delivery of alpha-1 antitrypsin.Research in pharmaceutical sciencesArticle
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Since the condition was first described four decades ago, alpha-1-antitrypsin (A1AT) deficiency has served as a model for other disease processes. A1AT is the archetypal serpin designed to ensnare proteases, a process that involves significant conformational change within the molecule. Mutations in the A1AT gene lead to misfolding of the protein and accumulation within the endoplasmic reticulum of hepatocytes resulting in two different pathologic processes. First, the accumulation of mutant A1AT protein has a directly toxic effect on the liver, resulting in hepatitis and cirrhosis. Second, the resultant decrease in circulating A1AT results in protease-antiprotease imbalance at the lung surface and emphysema ensues. A1AT deficiency therefore can be seen as two distinct disease processes: a conformational disease of the liver and a protease-antiprotease imbalance of the lung. This two-stage model of disease in A1AT deficiency is elegant in its simplicity and goes a long way to explaining the clinical manifestations that occur in patients with the condition. However, some aspects of the disease are not readily explained. Recent findings suggest that there is more to the lung damage in A1AT deficiency than simple proteolytic insult and that the presence of the mutant protein itself is proinflammatory and may indeed cause chronic injury to the cells that produce it. This review discusses some of the emerging concepts in alpha-1-antitrypsin research and outlines the implications these new ideas may have for treatment of this condition.
Indexed as
Identifiers
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.