Molecular Basis of Pulmonary Disease : Insights from Rare Lung Disorders

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Molecular Basis of Pulmonary Disease : Insights from Rare Lung Disorders Description

One premise of this book is that the study of rare lung disorders enhances our understanding of common pulmonary diseases and disease mechanisms such as fibrosis and emphysema. For example, the finding that the mutations in the alveolar type II cell specific protein, SP-C, can lead to pulmonary fibrosis is prima facie evidence that the alveolar epithelium plays a critical role in fibrogenesis. Similiarly, development of premature emphysema in patients with alpha one antitrypsin deficiency provides strong support for the theory of protease/protease inhibitior balance in the pathogenesis of alveolar destruction. A second equally important premise is that the science in rare lung disease is inherently [Read more...]

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