Mechanisms of emphysema in alpha1-antitrypsin deficiency: molecular and cellular insights.

@article{Gooptu2009MechanismsOE,
  title={Mechanisms of emphysema in alpha1-antitrypsin deficiency: molecular and cellular insights.},
  author={Bibek Gooptu and Ugo I. Ekeowa and David A. Lomas},
  journal={The European respiratory journal},
  year={2009},
  volume={34 2},
  pages={475-88}
}
The severe, early onset emphysema that occurs in patients with circulating deficiency of alpha(1)-antitrypsin (alpha(1)-AT) attests to the importance of this protease inhibitor in maintaining lung parenchymal integrity. It has led to the powerful concept of protease:antiprotease balance being crucial to alveolar homeostasis. Pathogenic mutations cause alpha(1)-AT to self-associate into polymer chains that accumulate intracellularly rather than proceeding along the secretory pathway… CONTINUE READING
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