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α1-Antitrypsin deficiency • 4: Molecular pathophysiology
  1. D A Lomas,
  2. H Parfrey
  1. Respiratory Medicine Unit, Department of Medicine, University of Cambridge, Cambridge Institute for Medical Research, Cambridge CB2 2XY, UK
  1. Correspondence to:
    Professor D Lomas
    Cambridge Institute for Medical Research, Wellcome Trust/MRC Building, Hills Road, Cambridge CB2 2XY, UK; dal16cam.ac.uk

Abstract

The molecular basis of α1-antitrypsin deficiency is reviewed and is shown to be due to the accumulation of mutant protein as ordered polymers within the endoplasmic reticulum of hepatocytes. The current goals are to determine the cellular response to polymeric α1-antitrypsin and to develop therapeutic strategies to block polymerisation in vivo.

  • α1-antitrypsin deficiency
  • molecular pathophysiology
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