Cystic fibrosis (CF) is a lethal inherited disease caused by mutations in the CF
transmembrane conductance regulator (CFTR) gene, which result in impairment of CFTR …

More than 2000 mutations in the cystic fibrosis transmembrane conductance regulator
(CFTR) have been described that confer a range of molecular cell biological and functional …

Little is known about the relative importance of monocyte and tissue-resident macrophages
in the development of lung fibrosis. We show that specific genetic deletion of monocyte …

Standard follow-up and symptomatic treatment have allowed most patients with cystic
fibrosis to live to young adulthood. However, many patients still die prematurely from …

Advances over the past 25 years have revealed much about how the structural properties of
membranes and associated proteins are linked to the thermodynamics and kinetics of …

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) gene that impair the function of CFTR, an epithelial chloride channel required for …

Diabetic nephropathy (DN) is among the most lethal complications that occur in type 1 and
type 2 diabetics. Podocyte dysfunction is postulated to be a critical event associated with …

Inflammation is a defensive reaction for external stimuli to the human body and generally
accompanied by immune responses, which is associated with multiple diseases such as …

Protein folding is a complex, error-prone process that often results in an irreparable protein
by-product. These by-products can be recognized by cellular quality control machineries …

Deletion of phenylalanine 508 of the cystic fibrosis transmembrane conductance regulator (∆
F508 CFTR) is the major cause of cystic fibrosis, one of the most common inherited …