Latest information on COVID-19
Support teaching, research, and patient care.
I began my career as a neuroscientist studying how neural circuits produce behavior, but changed interests in mid-career to study cystic fibrosis (CF), with the goal of ameliorating the symptoms in people who have CF. I direct the Cystic Fibrosis Research Laboratory at Stanford. We discovered that a specific kind of sweating is rate-limited by CFTR--the anion channel product of the CF gene. We demonstrated that airway glands, which produce antibiotic-rich mucus that helps protect the airways, display a profound secretory defect in cystic fibrosis. Current research uses sweat secretion as a sensitive assay of CFTR function that can be used to assess the efficacy of drugs that improve CFTR function. We also study airway mucociliary clearance, and promote a preventative approach to lung infections in people with CF.
The goal is to understand how a defective ion channel leads to the human genetic disease cystic fibrosis. Studies of ion channels and ion transport involved in gland fluid transport. Methods include SSCP mutation detection and DNA sequencing, protein analysis, patch-clamp recording, ion-selective microelectrodes, electrophysiological analyses of transmembrane ion flows, isotopic metho
(Study: Vertex IIS) Does Ivacaftor Alter Wild Type CFTR-Open Probability In The Sweat Gland Secretory Coil?
Clinical studies of lumacaftor + ivacaftor (combo therapy) produced better FEV1 (forced
expiratory volume in 1 second) improvements than ivacaftor alone, without further improvement
in sweat chloride results.
To help understand why sweat chloride was unresponsive, the investigators will use a newly
developed sweat secretion test that provides accurate, in vivo readout of CFTR (cystic
fibrosis transmembrane conductance regulator) function in the sweat gland secretory coil.
The investigators devised a protocol to determine if short courses of ivacaftor (3.5 days)
will produce significant increases in WT (Wild-Type, i.e. normal) CFTR open probability by
measuring CFTR-dependent sweating (C-sweat) in subjects with WT CFTR.
Stanford is currently not accepting patients for this trial.
For more information, please contact Colleen Dunn, RRT, CCRC, 650-736-0388.
View full details