Accumulation of a fatty compound called ceramide in the lungs could set the stage for the chronic lung infections of cystic fibrosis, a study in mice suggests. The finding offers a new twist on the still-unresolved course of this hereditary disease.
Patients with cystic fibrosis have a mutation in the CFTR gene. The protein normally encoded by this gene shuttles ions in and out of cells, ushering needed chemicals through the cell's membrane. But because cystic fibrosis patients' CFTR protein is missing or defective, the shuttling goes awry and sets off a process that leaves patients' lungs cluttered with mucus. The ultimate result is the respiratory failure characteristic of this disease.
Earlier studies had shown that a faulty or missing CFTR protein could make cells more alkaline. In the new study, physician Erich Gulbins of the University of Duisburg-Essen in Germany and