&agr;1-Antitrypsin Deficiency:: Biological Answers to Clinical Questions

@article{Coakley2001agr1AntitrypsinDB,
  title={\&agr;1-Antitrypsin Deficiency:: Biological Answers to Clinical Questions},
  author={Raymond J. Coakley and Clifford C Taggart and Shane J. O’Neill and Noel G. McElvaney},
  journal={The American Journal of the Medical Sciences},
  year={2001},
  volume={321},
  pages={33-41}
}
&agr;1-antitrypsin (&agr;1AT) deficiency is a common lethal hereditary disorder of white persons of European descent. The condition is characterized by reduced serum levels of &agr;1AT, a 52-kDa glycoprotein synthesized chiefly in the liver and, to a lesser extent, by macrophages and neutrophils. &agr;1AT acts as an antiprotease and is the physiological inhibitor of neutrophil serine proteases such as neutrophil elastase cathepsin G and proteinase 3. The clinical manifestations of &agr;1AT… 
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Diagnostic methods are summarised in table 1 and associations with á-1 antitrypsin deficiency in adult life are associations with Membranoproliferative glomerulonephritis, panniculitis, and necrotising vasculitis.
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TLDR
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TLDR
There is no relationship between alpha1-AT deficient genotypes S and Z and lung cancer in patients involved in this study, and all lung cancer patients were of M genotype, and no Z or S genotypes were detected.
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An overview of the roles that a1-antitrypsin may play in atherogenesis is provided, the findings from exercise studies in which it was measured are summarised, and it is proposed that transient exercise induced elevations in a1- Antitriesin may potentially contribute to the anti-inflammatory effect of exercise.
Alpha 1-Antitrypsin Inhibitor Therapy
  • Medicine, Biology
  • 2019
TLDR
AAT inhibitor therapy is considered not medically necessary for use in individuals with the PiMZ or PiMS phenotypes of AAT deficiency because these individuals appear to be at small risk of developing panacinar emphysema.
Z alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant.
TLDR
It is suggested that the polymerization of locally produced ZA1AT is a contributory factor to the lung inflammation experienced by those with A1AT deficiency and that standard antiprotease therapies may not address this problem.
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