Elastin Peptides as a Potential Disease Vector in the Pathogenesis of Pulmonary Emphysema: An Investigation of This...

The degradation of elastic fibers is a fundamental characteristic of pulmonary emphysema, resulting in the release of proinflammatory elastin peptides. The findings discussed in this paper support the hypothesis that these peptides act as carriers of disease, interacting with elastin receptor complexes that promote inflammation, elastic fiber damage, and airspace enlargement. Studies from our laboratory show that the breakdown of these fibers is significantly enhanced by intratracheal instillation of elastin peptides in a lipopolysaccharide-induced model of acute lung injury. This result is consistent with a mechanism of elastic fiber injury in which an expanding pool of elastin peptides generates further elastolysis. The accelerating release of the peptides results in a self-perpetuating disease process with the features of an epidemic, where self-replicating agents spread disease. As in the case of an epidemic, elastin peptides resemble disease vectors that transmit alveolar wall injury throughout the lung. This concept may provide a framework for developing novel therapeutic approaches specifically designed to protect elastic fibers from various enzymatic and oxidative insults, thereby slowing the progression of a disease with no robust treatment options.