Cystic  Fibrosis 
    Research  Laboratory
    Phone: (650) 725-2785 or 2468  
    last edited 03/08/2023
    Stanford University 
    Bldg 420, Jane Stanford Way
    Stanford, CA, 94305-2130
    Mission: CFRL carries out basic research designed to ameliorate cystic fibrosis (CF).  We have 3 main research projects. 
    1. To understand and help eliminate CF lung infections. Our hypothesis is that CF lungs will retain near normal function if infections can be held at bay.  We further hypothesize that the susceptibility to lung infections is the result of abnormal airway mucus, which, because of the failure of CFTR-dependent, anion-mediated fluid secretion, is deficient in both its ability to arrest bacterial growth and to sweep bacteria from the airways.
    2.  To quantify the amount of CFTR function in people with and without CF, and in people taking CFTR-directed therapeutics, such as ivacaftor.  We do this by measuring sweat rates from individual, identified sweat glands, as shown in the figure. We have used these measures to show that carriers produce 1/2 the normal level of sweat when elicited by a method that is dependent upon CFTR. Using the method, we have recalibrated the relation of sweat chloride levels to level of CFTR activity, and find that most current calibrations overestimate CFTR activity in carriers and CF subjects taking CFTR modulators.
    3. To provide a therapy that can be useful now in all CF subjects, regardless of their mutation, we are developing an inhaled therapy based on our discovery that profound, synergistic increases in the velocity of mucocililary clearance can by achieved combining beta-adrenergic and low dose cholinergic drugs. Importantly, these increases are partially preserved in CF airway ex vivo animal models. We are working to bring this regimen to the clinic, where we expect it will offer considerable therapeutic benefit in diseases with mucus obstruction.
    Wine Home
    		                                  

    		Please see Jeffrey Wine Profile for current publication list.