Reversing lipid woes in cystic fibrosisMar. 3, 2012, 9:55 AM
Patients with cystic fibrosis (CF) – the most commonly inherited genetic disorder among Caucasians – have abnormalities in blood and tissue polyunsaturated fatty acid (PUFA) levels. In mouse models of CF, supplementation with the fatty acid DHA can reverse the PUFA abnormalities and CF-related pathologies.
Adam Seegmiller, assistant professor of pathology, and colleagues previously found in a cell culture model of CF that PUFA abnormalities correlated with increased expression and activity of certain fatty acid metabolic enzymes called desaturases. They have now demonstrated that addition of DHA or the fatty acid EPA to the cells reduces the expression and activity of these desaturases and reverses the PUFA abnormalities. They also uncovered other metabolic abnormalities in the CF cells, including increased fatty acid uptake and increased conversion of DHA to EPA.
The results, reported in the February Journal of Lipid Research, indicate that the fatty acid abnormalities of CF are related to alterations of PUFA metabolism – and that they may be reversed by supplementation with DHA and EPA.
This research was funded in part by the Edward and Nancy Fody Endowed Chair in Pathology and by the Vanderbilt Physician Scientist Training Program.