Biochemistry Of Pulmonary Emphysema (current Topics In Rehabilitation)

Pulmonary emphysema is a disease which develops because of a localized imbalance between endogenous proteinase inhibitors and proteinases leaking from neurophils during phagocytosis at inflammatory foci within the lung. This volume not only reviews at a biochemical level what is known about the natural inhibitors and proteinases involved in connective tissue destruction within the lung, but also suggests novel methodologies for reestablishing proper enzyme-inhibitor balance, including the use of natural or synthetic inhibitors for supplementation or gene therapy. Front Matter....Pages I-XV Pulmonary Emphysema: What’s Going On....Pages 1-11 Elastin and the Lung....Pages 13-25 An Introduction to the Endopeptidases....Pages 27-34 Lung Proteinases and Emphysema....Pages 35-46 Proteinases and Proteinase Inhibitors in the Pathogenesis of Pulmonary Emphysema in Humans....Pages 47-69 Multiple Functions of Neutrophil Proteinases and their Inhibitor Complexes....Pages 71-79 Kinetics of the Interaction of Human Leucocyte Elastase with Protein Substrates: Implications for Enzyme Inhibition....Pages 81-100 Proteinase Inhibitor Candidates for Therapy of Enzyme-Inhibitor Imbalances....Pages 101-112 Antileucoprotease (Secretory Leucocyte Proteinase Inhibitor), a Major Proteinase Inhibitor in the Human Lung....Pages 113-121 Synthetic Mechanism-Based and Transition-State Inhibitors for Human Neutrophil Elastase....Pages 123-141 Development and Evaluation of Antiproteases as Drugs for Preventing Emphysema....Pages 143-158 Genetic Control of Human Alpha-1-Antitrypsin and Hepatic Gene Therapy....Pages 159-167 Neutrophils, Neutrophil Elastase and the Fragile Lung: The Pathogenesis and Therapeutic Strategies Relating to Lung Derangement in the Common Hereditary Lung Disorders....Pages 169-187 Workshop Summary....Pages 189-192 Back Matter....Pages 193-200