Alpha-1-Antitrypsin Deficiency

  • Diane Wilson Cox
Part of the Hepatology book series (H, volume 5)


Alpha-1-antitrypsin (α1AT) is one of several protease inhibitors in serum which together control proteases involved in such vital processes as activation of the clotting system, activation and control of the complement system, removal of dead cells and tissues, and possibly control of the immune response. α1AT is the most abundant of the serum protease inhibitors on a molar basis and inhibits a wide spectrum of proteases including leukocyte proteases, thrombin, and bacterial proteases. This serum protein is particularly interesting from a genetic viewpoint in that it illustrates how a genetic predisposition can interact with other factors (genetic or environmental), to produce disease.


Liver Disease Biliary Atresia Severe Combine Immune Deficiency Neonatal Hepatitis Extrahepatic Cholestasis 
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Copyright information

© Plenum Press, New York 1983

Authors and Affiliations

  • Diane Wilson Cox
    • 1
    • 2
  1. 1.Research InstituteThe Hospital for Sick ChildrenCanada
  2. 2.Departments of Paediatrics, Medical Genetics and Medical BiophysicsUniversity of TorontoCanada

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