LL02--Alveoli: A Player in the CF Lung?

Pulmonary manifestations are a primary cause of morbidity and mortality in pwCF. Most features of CF lung pathology are associated with defective CFTR function which disrupts the innate defense of the mucosal surfaces of the conducting airways. Recently, sensitive molecular methods have better defined the features of individual single cells within the lung. These methods have identified the major cell types expressing CFTR in the lung, which includes a relatively high expression of CFTR mRNA in alveolar type II pneumoctyes (AT2). Despite high CFTR expression in AT2 cells of the non-CF lung, the alveolar regions of the CF lung remain relatively healthy with little evidence of overt pathology; even in end-stage CF lung disease at the time of transplant. Why alveolar pneumocytes express CFTR and why an absence of CFTR function in the alveolus of pwCF does not result in obvious loss of lung function remains poorly understood. This lunch-and-learn session will explore this curious phenomenon and will seek input from the research community as to how to address this question, since ultimately it will be important for therapeutic development to know if defective CFTR function in AT2 cells in the CF lung will also require correction to achieve maximal therapeutic benefit.