Additional disruption of the ClC-2 Cl(-) channel does not exacerbate the cystic fibrosis phenotype of cystic fibrosis transmembrane conductance regulator mouse models.

Abstract excerpt

Cystic fibrosis is a fatal inherited disease that is caused by mutations in the gene encoding a cAMP-activated chloride channel, the cystic fibrosis transmembrane conductance regulator (CFTR). It has been suggested that the cystic fibrosis phenotype might be modulated by the presence of other Cl(-) channels that are coexpressed with CFTR in some epithelial cells. Because the broadly expressed plasma membrane...

Topics

• Animals
• CLC-2 Chloride Channels
• Cell Membrane
• Chloride Channels
• Colon
• Cystic Fibrosis
• Cystic Fibrosis Transmembrane Conductance Regulator
• Disease Models, Animal
• Epithelium
• Heterozygote
• Mice
• Mice, Inbred C57BL
• Mice, Knockout