Biochemistry, pathology, and genetics of pulmonary emphysema by Bignon, Jean; Scarpa, Giovanni Luigi

By Bignon, Jean; Scarpa, Giovanni Luigi

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Extra info for Biochemistry, pathology, and genetics of pulmonary emphysema : proceedings of an international symposium held in Sassari, Italy, 27-30 April 1980

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Dis. 1978; 118:617–621. 11. , JANOFF, A. In vitro, suppression of serum elastase-inhibitory capacity by reactive oxygen species generated by phagocytosing polymorphonuclear leukocytes. J. clin. Invest. 1979; 63:793–797. 12. , SANNA-RANDACCIO, A. L’emphysème expérimental. Bull, europ. Physiopath, resp. 1977; 13:561–590. 13. A. Irreversible bronchial goblet cell metaplasia in hamsters with elastase-induced panacinar emphysema. J. clin. Invest. 1977; 59:397–404. 14. Ciba Guest Symposium. — Terminology, definitions and classification of chronic pulmonary emphysema and related conditions.

These cells are probably implicated in the pathogenesis of emphysema in human lungs. b) Elastase inhibitors. Of the serum protease inhibitors so far identified, α1AP and α2-macroglobulin (α2M) appear to be most important. α2M is a large protein, of 800,000 daltons, which is restricted almost entirely to the vascular compartment and does not normally reach the alveolar spaces. No α2M deficiency has been observed in humans; however, the complexity of the interaction between elastase and α2M and α1AP may result in an increase in human leukocyte elastase activity in the presence of α2M when there is a deficiency of α1AP [24, 73].

Clin. Invest. 1977; 59:397–404. 14. Ciba Guest Symposium. — Terminology, definitions and classification of chronic pulmonary emphysema and related conditions. Thorax, 1959, 14, 286–299 15. B. α1-antitrypsin as a systemic determinant of lung structure and function. , ed. Lung Biology in Health and Disease, vol. 2: The biochemical basis of pulmonary function. New York: Dekker; 1976:313–360. 16. B. The effects in vivo, and in vitro, of oxidative damage to purified α1-antitrypsin and to the enzyme-inhibiting activity of plasma.

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