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.

Keywords::

• amitripyline
• ceramide
• Cftr
• cystic fibrosis
• Pseudomonas aeruginosa

Financial & competing interests disclosure

Studies performed by Katrin Anne Becker, Brian Henry, Regan Ziobro, Joachim Riethmüller and Erich Gulbins and described in the present review were supported by DFG grants GU 355/16-1 and GU 335/16-2. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.