[HTML][HTML] Glycerol Reverses the Misfolding Phenotype of the Most Common Cystic Fibrosis Mutation (∗)
The common ΔF508 mutation in the cystic fibrosis transmembrane conductance regulator
(CFTR) interferes with the biosynthetic folding of nascent CFTR polypeptides, leading to
their retention and rapid degradation in an intracellular compartment proximal to the Golgi
apparatus. Neither the pathway by which wild-type CFTR folds nor the mechanism by which
the Phe 508 deletion interferes with this process is well understood. We have investigated
the effect of glycerol, a polyhydric alcohol known to stabilize protein conformation, on the …
(CFTR) interferes with the biosynthetic folding of nascent CFTR polypeptides, leading to
their retention and rapid degradation in an intracellular compartment proximal to the Golgi
apparatus. Neither the pathway by which wild-type CFTR folds nor the mechanism by which
the Phe 508 deletion interferes with this process is well understood. We have investigated
the effect of glycerol, a polyhydric alcohol known to stabilize protein conformation, on the …