The Cystic Fibrosis ‘Gender Gap’: Past Observations Present Understanding and Future Directions

Sanjay H. Chotirmall, Catherine M. Greene, Brian J. Harvey & Noel G. McElvaney
Cystic Fibrosis (CF) is a systemic disease impacting upon several organ systems. These include gastrointestinal, reproductive, endocrine and pulmonary manifestations of which the latter contributes the heaviest burden of disease morbidity, mortality and impact on quality of life. The defective protein in the disease state is the Cystic Fibrosis Transmembrane Regulator (CFTR) that normally functions as an ion channel permitting intracellular chloride escape. Regulated by cyclic adenosine monophosphate (cAMP) and localized to the apical membrane...
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