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

  title={&agr;1-Antitrypsin Deficiency:: Biological Answers to Clinical Questions},
  author={R. Coakley and C. Taggart and S. O'Neill and N. Mcelvaney},
  journal={The American Journal of the Medical Sciences},
  • R. Coakley, C. Taggart, +1 author N. Mcelvaney
  • Published 2001
  • Medicine
  • The American Journal of the Medical Sciences
  • &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… CONTINUE READING
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