title: Lipids in cystic fibrosis.
abstract: Cystic fibrosis is characterized by severe intestinal and pulmonary symptoms, but also changes in the liver, pancreas and reproductive tract. Since gastrointestinal symptoms can be controlled, the quality of live and longevity of cystic fibrosis patients is mainly determined by pulmonary inflammation and chronic pulmonary infections with Pseudomonas aeruginosa, Staphylococcus aureus, Burkholderia cepacia and Haemophilus influenzae. Recent studies developed novel and exciting concepts regarding the pathogenesis and treatment of cystic fibrosis. In particular, several studies indicated a critical role of death receptors, caveolae proteins, membrane rafts, alterations of the ceramide metabolism with an accumulation of ceramide and a reduction of 15-keto-prostaglandin 2 in cystic fibrosis lungs. These alterations have been found to be critically involved in the pulmonary inflammation and infection susceptibility of cystic fibrosis patients. However, albeit these studies provided novel insights into molecular mechanisms causing inflammation and vulnerability to infection, the details of these processes are still unknown.
authors.affiliation: Department of Molecular Biology, University of Duisburg-Essen, Hufelandstrasse 55, 45122 Essen, Germany.
authors.forename: Katrin Anne
authors.initials: KA
authors.lastname: Becker
authors.forename: Brian
authors.initials: B
authors.lastname: Henry
authors.forename: Regan
authors.initials: R
authors.lastname: Ziobro
authors.forename: Joachim
authors.initials: J
authors.lastname: Riethmüller
authors.forename: Erich
authors.initials: E
authors.lastname: Gulbins
mesh_terms: D000818:Animals; D002518:Ceramides / Q000378:metabolism; D003550:Cystic Fibrosis / Q000276:immunology / Q000378:metabolism* / Q000382:microbiology; D004195:Disease Models, Animal; D006801:Humans; D050356:Lipid Metabolism; D008168:Lung / Q000276:immunology / Q000378:metabolism* / Q000382:microbiology
chemical_list: D002518:Ceramides