May 2005
Thorax;May2005, Vol. 60 Issue 5, p432
Academic Journal
The article presents information on a novel mechanism of emphysema pathogenesis in Z-Alpha-1-antitrypsin (AT) homozygotes. AT is the predominant proteinase inhibitor in the lung. There are many abnormal variants of AT and the Z form is one of the most frequently encountered. Z-AT is prone to polymerization and the polymeric form aggregates in the liver, causing a plasma deficiency, while monomeric Z-AT is a less effective proteinase inhibitor than normal AT. This quantitative and functional deficiency exposes the lung to damaging proteinases such as neutrophil elastase which results in emphysema.


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