CFTR activity modulates mitochondrial fragmentation and affects mitochondrial complex I activity in cystic fibrosis airway ephitelial cells

VALDIVIESO, ANGEL GABRIEL; CLAUZURE, MARIÁNGELES; MASSIP-COPIZ, MARÍA M; MORI, CONSUELO; SANTA COLOMA, TOMÁS A.

Mar del Plata

Congreso; LXIII Reunión Anual de la Sociedad Argentina de Investigación Clínica - SAIC.; 2018

Sociedad Argentina de Investigación Clínica

Mitochondria are dynamic organelles that continuously join (fusion) and divide (fission), regulating cellular homeostasis. CFTR is the gene responsible for Cystic Fibrosis (CF) disease and encodes a cAMP-activated chloride (Cl-) channel, causing a variety of alter­ations such as differential gene expression and mitochondrial dysfunction. lmpairment of the CFTR channel activity causes a de­creased mitochondrial complex-I activity (mCx-I) and an increased ROS production. CFTR activity could regulate mitochondrial func­tion through the modulation of mitochondrial dynamics, explaining mitochondrial defects observed in CF. The mitochondrial network morphology was studied in the bronchial epithelial IB3-1 cells (CF) and compared with IB3-1 CFTR corrected cells (S9). Cells incubated for 24 h in serum-free medium were stained with the fluorescent mitochondrial probe TMRE and analyzed by confocal microscopy. Small mitochondria population was significantly increased (p