[CITATION][C] The cystic fibrosis transmembrane conductance regulator
JR Riordan - Annual review of physiology, 1993 - annualreviews.org
Cystic fibrosis (CF) is a fatal genetic disease with an especially high incidence in Caucasian
populations, where it has a frequency of approximately one in 2500 live births (11). There is
dysfunction at wet epithelial surfaces of a range of tissues, but most significantly those of the
gastrointestinal and respiratory tracts. It is widely accepted that as a consequence of an
aberrant control of salt and water movement across these surfaces, macromolecular
secretions have an altered physical state and cannot be normally cleared (27). This results …
populations, where it has a frequency of approximately one in 2500 live births (11). There is
dysfunction at wet epithelial surfaces of a range of tissues, but most significantly those of the
gastrointestinal and respiratory tracts. It is widely accepted that as a consequence of an
aberrant control of salt and water movement across these surfaces, macromolecular
secretions have an altered physical state and cannot be normally cleared (27). This results …
