Increased expression of ATP12A proton pump in cystic fibrosis airways

• DOI: 10.1172/jci.insight.123616
• Publication Date: 2018-10-17T15:01:49Z
• Authors: Paolo Scudieri; Ilaria Musante; Emanuela Caci; Arianna Venturini; Patrizia Morelli; Christine Walter; Davide Tosi; Alessandro Palleschi; Pablo Martin-Vasallo; Isabelle Sermet-G...; Gabrielle Planelles; Gilles Crambert; Luis J.V. Galietta
• Keywords: Submucosal glands; Bicarbonate; Basal (medicine); Apical membrane

Proton secretion mediated by ATP12A protein on the surface of airway epithelium may contribute to cystic fibrosis (CF) lung disease favoring bacterial infection and obstruction. We studied in fresh bronchial samples cultured epithelial cells. In vivo, expression was found almost exclusively at apical side nonciliated cells submucosal glands, with much higher CF samples. This could be due inflammation, since treating supernatants or IL-4 (a cytokine that induces goblet cell hyperplasia) increased observation associated upregulation translocation ATP1B1 from basal side, where it colocalizes ATP12A. function evaluated measuring pH fluid epithelia. Under resting conditions, epithelia showed more acidic values. abnormality minimized inhibiting ouabain. Following treatment IL-4, markedly increased, as indicated strong acidification occurring under bicarbonate-free conditions. Our study reveals potentially novel aspects remarks its importance a possible therapeutic target other respiratory diseases.