title: Is cystic fibrosis a disease confined to epithelial cells?
abstract: We have reviewed evidence regarding the basic tissue involvement in cystic fibrosis (CF). CF is a disease almost exclusively confined to epithelial tissues, particularly those of endodermal origin. Exocrine glands containing such epithelium typically show both obstructive and electrolyte secretory defects. To date most basic CF research has centered on study of mesodermally-derived tissues in which little pathology is seen. The focus of CF research should be changed to concentrate on epithelial tissues.
mesh_terms: D003550:Cystic Fibrosis / Q000473:pathology*; D004847:Epithelial Cells; D006801:Humans; D007421:Intestine, Small / Q000473:pathology; D009092:Mucous Membrane / Q000473:pathology; D009928:Organ Specificity; D010179:Pancreas / Q000473:pathology; D012469:Salivary Glands / Q000473:pathology