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Department of Physiology and Pharmacology, University of Queensland, St. Lucia, Queensland 4072, Australia
More than 1,300 different mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) are the cause for cystic fibrosis. CFTR is in charge of proper secretion and absorption of electrolytes, and thus the disease is characterized by defective epithelial Cl secretion and enhanced Na+ absorption. Recent studies show that CFTR interacts with other proteins via PDZ domains.
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