The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-regulated
chloride (Cl−) channel. Mutations of its gene lead to the disease of cystis fibrosis (CF) …

Cystic fibrosis (CF), a fatal genetic disorder predominant in the Caucasian population, is
caused by mutations in the cystic fibrosis transmembrane conductance regulator (Cftr) gene …

Inflammatory lung disease is the major cause of morbidity and mortality in cystic fibrosis
(CF); understanding what produces dysregulated innate immune responses in CF cells will …

The organization of respiratory chain complexes in supercomplexes has been shown in the
mitochondria of several eukaryotes and in the cell membranes of some bacteria. These …

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) is a chloride channel found
in secretory epithelia with a plethora of known interacting proteins. Mutations in the CFTR …

Misfolded F508del-CFTR, the main molecular cause of the recessive disorder cystic fibrosis,
is recognized by the endoplasmic reticulum (ER) quality control (ERQC) resulting in its …

Respiratory diseases affect humanity globally, with chronic lung diseases (eg, asthma,
chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, among others) and …

The most prevalent cause of cystic fibrosis (CF) is the deletion of a phenylalanine residue at
position 508 in CFTR (ΔF508-CFTR) protein. The mutated protein fails to fold properly, is …

Proteomic analysis has proved to be an important tool for understanding the complex nature
of genetic disorders, such as cystic fibrosis (CF), by defining the cellular protein environment …

Cystic Fibrosis (CF) is a recessively inherited disease caused by mutations in the Cystic
Fibrosis Transmembrane Conductance Regulator (CFTR) gene. CFTR has a pivotal role in …