The basic defect in cystic fibrosis.

J H Widdicombe; J J Wine

doi:10.1016/0968-0004(91)90183-v

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The basic defect in cystic fibrosis.

J H Widdicombe·J J Wine

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Abstract

Recent evidence strongly suggests that the cystic fibrosis gene product (CFTR) is a Cl- channel. Its properties, however, differ from those of a 30-50 pS outwardly rectifying channel previously implicated as defective in cystic fibrosis. It is still uncertain whether the pleiotropic effects of the CF defect, such as increased airway Na+ absorption and mucus sulfation, are secondary to reduced Cl- conductance, or reflect additional functions of CFTR.

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Chloride Channels Chlorides Cystic Fibrosis Cystic Fibrosis Transmembrane Conductance Regulator Humans Ion Channels Membrane Proteins Sodium Journal Article Review

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J H Widdicombe, J J Wine. 1991. The basic defect in cystic fibrosis.. https://doi.org/10.1016/0968-0004(91)90183-v

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