Impaired Renal HCO₃ ^- Excretion in Cystic Fibrosis.

ABSTRACT:

Background

Patients with cystic fibrosis (CF) do not respond with increased urinary HCO₃ ^- excretion after stimulation with secretin and often present with metabolic alkalosis.

Methods

By combining RT-PCR, immunohistochemistry, isolated tubule perfusion, in vitro cell studies, and in vivo studies in different mouse models, we elucidated the mechanism of secretin-induced urinary HCO₃ ^- excretion. For CF patients and CF mice, we developed a HCO₃ ^- drinking test to assess the role of the cystic fibrosis transmembrane conductance regulator (CFTR) in urinary HCO₃ ^-excretion and applied it in the patients before and after treatment with the novel CFTR modulator drug, lumacaftor-iv

SUBMITTER: Berg P 

PROVIDER: S-EPMC7460909 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature