The mechanisms remain uncertain by which mutations in CFTR cause lung disease in cystic
fibrosis (CF). Teichgräber et al. recently reported increased ceramide in CF lungs, which
was proposed to result from defective lysosomal acidification in airway epithelial cells and
consequent impairment of pH-dependent ceramide-metabolizing enzymes (Teichgräber,
V., Ulrich, M., Endlich, N., Reithmüller, J., Wilker, B., Conceição Ce Olivereira-Munding,
C., van Heeckeren, AM, Barr, ML, von Kürthy, G., Schmid, KW, Weller, M., Tümmler, B …