The Cl−-transporting proteins CFTR, SLC26A9, and anoctamin (ANO1; ANO6) appear to
have more in common than initially suspected, as they all participate in the pathogenic …

Triple combination therapy with the CFTR modulators elexacaftor (ELX), tezacaftor (TEZ)
and ivacaftor (IVA) has been qualified as a game changer in cystic fibrosis (CF). We provide …

The kidney plays a crucial role in acid-base homeostasis. In the distal nephron, α-
intercalated cells contribute to urinary acid (H+) secretion and β-intercalated cells …

Abstract Pendrin (SLC26A4) is an anion exchanger from the SLC26 transporter family which
is mutated in human patients affected by Pendred syndrome, an autosomal recessive …

Background: Elexacaftor-Tezacaftor-Ivacaftor (ELE/TEZ/IVA) is believed to be an effective
and well-tolerated treatment for cystic fibrosis (CF), but the exact efficacy and safety profile …

Background The secretin receptor (SCTR) is functionally expressed in the basolateral
membrane of the β-intercalated cells of the kidney cortical collecting duct and stimulates …

Secretin is a key hormone of the intestinal phase of digestion which activates pancreatic,
bile duct and Brunner gland HCO3− secretion. Recently, the secretin receptor (SCTR) was …

Methods: 24-hour urine collections were included from three clinical studies of patients with
CKD stage 3 and 4: A development cohort (n= 82), a variation cohort (n= 58), and a …

Abstract pH is one of the key parameters in chemistry and impacts almost all chemical and
biological processes. Also, within analytical chemistry and sensing, pH plays a critical role …

Aim Renal excretion of excess HCO3− depends on renal cystic fibrosis transmembrane
conductance regulator (CFTR) and is impaired in people with cystic fibrosis (pwCF). Urine …