The epithelial sodium channel (ENaC) is composed of three homologous subunits and
allows the flow of Na+ ions across high resistance epithelia, maintaining body salt and water …

Bronchiectasis is a complex, heterogeneous disorder defined by both a radiological
abnormality of permanent bronchial dilatation and a clinical syndrome. There are multiple …

Background Around 20% of people with cystic fibrosis (pwCF) do not have access to the
triple combination elexacaftor/tezacaftor/ivacaftor (ETI) in Europe because they do not carry …

Defective transepithelial electrolyte transport is thought to initiate cystic fibrosis (CF) lung
disease. Yet, how loss of CFTR affects electrolyte transport remains uncertain. CFTR−/− pigs …

Introduction: Cystic fibrosis is an autosomal recessive disorder caused by mutations in the
cystic fibrosis transmembrane conductance regulator (CFTR) gene that codes for the CFTR …

Epithelial sodium channels (ENaC) are part of a complex network of interacting biochemical
pathways and as such are involved in several disease states. Dependent on site and type of …

Sodium transport in the distal nephron is mediated by epithelial sodium channel activity.
Proteolytic processing of external domains and inhibition with increased sodium …

In the majority of cases, there is no difficulty in diagnosing Cystic Fibrosis (CF). However,
there may be wide variation in signs and symptoms between individuals which encourage …

The positional cloning of the gene responsible for cystic fibrosis (CF) was the important first
step in understanding the basic defect and pathophysiology of the disease. This study aims …

Liddle syndrome is an autosomal dominant form of hypokalemic hypertension due to
mutations in the β-or γ-subunit of the epithelial sodium channel (ENaC). Here, we describe a …