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

VALDIVIESO ANGEL GABRIEL; CLAUZURE M; C. MORI; MASSIP-COPIZ, M.; SANTA COLOMA TA,

Congreso; LXIII Reunión de la Sociedad Argentina de Investigación Clínica (SAIC); 2018

CFTR ACTIVITY MODULATES MITOCHONDRIALFRAGMENTATION AND AFFECTS MITOCHONDRIALCOMPLEX I ACTIVITY IN CYSTIC FIBROSIS AIRWAYEPHITELIAL CELLSAngel Gabriel Valdivieso1, Mariangeles Clauzure1, María MacarenaMassip Copiz1, Consuelo Mori1, Tomás Santa Coloma11Investigaciones Biomédicas (BIOMED) UCA-CONICETMitochondria are dynamic organelles that continuously join (fusion)and divide (fission), regulating cellular homeostasis. CFTR is thegene responsible for Cystic Fibrosis (CF) disease and encodes acAMP-activated chloride (Cl-) channel, causing a variety of alterationssuch as differential gene expression and mitochondrial dysfunction.Impairment of the CFTR channel activity causes a decreasedmitochondrial complex-I activity (mCx-I) and an increasedROS production. CFTR activity could regulate mitochondrial functionthrough the modulation of mitochondrial dynamics, explainingmitochondrial defects observed in CF. The mitochondrial networkmorphology was studied in the bronchial epithelial IB3-1 cells (CF)and compared with IB3-1 CFTR corrected cells (S9). Cells incubatedfor 24 h in serum-free medium were stained with the fluorescentmitochondrial probe TMRM and analyzed by confocal microscopy.Small mitochondria population was significantly increased (p