Cystic fibrosis (CF), the most common lethal genetic disease in the Caucasian population, is
caused by loss-of-function mutations of the CF transmembrane conductance regulator …

All cellular proteins are subject to quality control" decisions," which help to prevent or delay
a myriad of diseases. Quality control within the secretory pathway creates a special …

Cystic fibrosis (CF) is a fatal genetic disease caused by mutations in the CFTR (cystic
fibrosis transmembrane conductance regulator) gene, which regulates chloride and water …

The cystic fibrosis transmembrane conductance regulator (CFTR) acts as a channel on the
apical membrane of epithelia. Disease-causing mutations in the cystic fibrosis gene can …

The folding and misfolding mechanism of multidomain proteins remains poorly understood.
Although thermodynamic instability of the first nucleotide-binding domain (NBD1) of ΔF508 …

Misfolding of ΔF508 cystic fibrosis (CF) transmembrane conductance regulator (CFTR)
underlies pathology in most CF patients. F508 resides in the first nucleotide-binding domain …

Background Long-term benefits of newborn screening (NBS) for cystic fibrosis (CF) have
been established with respect to nutritional status, but effects on pulmonary health remain …

In this review, we describe our current understanding of translation termination and
pharmacological agents that influence the accuracy of this process. A number of drugs have …

An attractive strategy to treat proteinopathies (diseases caused by malformed or misfolded
proteins) is to restore protein function by inducing proper three-dimensional structure. We …

Since the discovery of the gene associated with cystic fibrosis (CF), there has been
tremendous progress in the care of patients with this disease. New therapies have entered …