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

  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},
  volume={34 2},
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
Related Discussions
This paper has been referenced on Twitter 1 time. VIEW TWEETS

From This Paper

Topics from this paper.


Publications citing this paper.
Showing 1-10 of 34 extracted citations

Clinical decision support system for chronic obstructive pulmonary disease using machine learning techniques

2017 International Conference on Electrical, Electronics, Communication, Computer, and Optimization Techniques (ICEECCOT) • 2017
View 1 Excerpt

Similar Papers

Loading similar papers…