[CFTR protein and molecular mechanisms of pulmonary involvement in cystic fibrosis].

Abstract excerpt

Cystic fibrosis is an often fatal hereditary disease mainly affecting the epithelium, especially in the airways, the pancreatic ducts, the sudoriparous glands and bile ducts. The pathophysiologic mechanism is complex but involves abnormal epithelial ion transport which controls water movement through the epithelial layers. In the bronchi, there is deficient secretion of chloride activated by cyclic AMP and...

Topics

• Biological Transport, Active
• Cystic Fibrosis
• Cystic Fibrosis Transmembrane Conductance Regulator
• Humans
• Ion Transport
• Lung Diseases
• Mutation