[Genetics and cellular biology of cystic fibrosis].

Abstract excerpt

Cystic fibrosis (CF) is the most frequent lethal genetic disease in caucasians. It behaves as an autosomal recessive disease. The CF gene encodes a protein named CFTR (for "Cystic Fibrosis Transmembrane conductance Regulator") which exerts multiple functions. One of the most important function is the regulation of sodium and chlorine transport in epithelia. Defects in this control induce dehydration of airway...

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