Epithelial vectorial ion transport in cystic fibrosis: Dysfunction, measurement, and pharmacotherapy to target the primary deficit

Cystic fibrosis patients display multi-organ system dysfunction (e.g. pancreas, gastrointestinal tract, and lung) with pathogenesis linked to a failure of Cl − secretion from the epithelial surfaces of these organs. If unmanaged, organ dysfunction starts early and patients experience chronic respira...

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Bibliographic Details
Main Authors: Lucy A Clunes, Naia McMillan-Castanares, Neil Mehta, Afia Mesadieu, Jorge Rodriguez, Mary Maj, Mark T Clunes
Format: Article
Language:English
Published: SAGE Publishing 2020-06-01
Series:SAGE Open Medicine
Online Access:https://doi.org/10.1177/2050312120933807