Molecular Basis of Pulmonary Disease

About this book

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 interesting and can often change with surprising speed. This is partly related to the fact that the genetic basis of many rare lung diseases is now available, providing a unique vantage point for framing research questions.

Written for: Pulmonologists, investigators, and basic scientists

Table of contents

1. Protein Misfolding
2. Aberrant Signaling
3. Intracellular Trafficking
4. Surfactant Function
5. Angiogenesis/Lymphangiogenesis
6. Airway Cell Function
7. Channel and Transport Dysfunction
8. Enzymatic Dysfunction
9. Antibody Mediated Lung Diseases
10. Sleep Disorders
11. Rare Lung Diseases of Unknown or Unclear Etiology
12.Facilitating Research in Rare Lung Disease