MODULATION OF PH BY EGFR IN CYSTIC FIBROSIS CELLS

MASSIP-COPIZ, MARÍA M.; MORI, CONSUELO; CLAUZURE, MARIÁNGELES; SANTA-COLOMA, TOMÁS A.; VALDIVIESO, ÁNGEL G.; ASENSIO, CRISTIAN J. A.

Mar del Plata

Congreso; LXIV Reunión Anual de la Sociedad Argentina de Investigación Clínica - SAIC; 2019

Abstract/Resumen: Cystic fibrosis (CF) is an autosomal recessive disease characterized by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene. The pulmonary parenchyma is affected severely by a vicious cycle of persistent inflammation and infections. Several hypotheses tried to explain the high susceptibility to lung infections in CF patients, including a reduction in the airway surface liquid (ASL) pH. A reduced bicarbonate transport through CFTR and an increased lactic acid secretion could explain the changes in extracellular pH. The aim of the present work was to study if the EGFR pathway is involved in the pH regulation in CF cells. We use two cellular models: IB3-1 cells (bronchial epithelial cells derived from a CF patient with a deltaF508/W1282X CFTR genotype) and C38 cells (IB3-1 cells transduced with an AAV vector to stably express a functional truncated CFTR version). The results obtained suggested that CFTR modulates significantly (p