Mutant (delta F508) cystic fibrosis transmembrane conductance regulator Cl- channel is functional when retained in endoplasmic reticulum of mammalian cells.

Abstract excerpt

Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR), a plasma membrane-localized chloride channel. Some mutations in CFTR, including one which affects most patients (delta F508-CFTR), prevent CFTR from exiting the endoplasmic reticulum (ER) where it is synthesized. To examine whether normal and mutant CFTRs function as chloride channels when...

Topics

• Animals
• CHO Cells
• Cell Nucleus
• Cells, Cultured
• Chloride Channels
• Cricetinae
• Cystic Fibrosis
• Cystic Fibrosis Transmembrane Conductance Regulator
• Endoplasmic Reticulum
• Humans
• Membrane Potentials