Mitochondrial Stress Responses and "Mito-Inflammation" in Cystic Fibrosis.

ABSTRACT: Cystic fibrosis (CF) is a genetic disease associated to mutations in the cystic fibrosis transmembrane conductance regulator gene, which results in the alteration of biological fluid and electrolyte homeostasis. The characteristic pathological manifestation is represented by exaggerated proinflammatory response in lung of CF patients, driven by recurrent infections and worsen by hypersecretion of proinflammatory mediators and progressive tissue destruction. Treating inflammation remains a priority in CF. However, current anti-inflammatory treatments, including non-steroidal agents, are poorly effective and present dramatic side effects in CF patients. Different studies suggest an intimate relationship between mitochondria and CF lung disease, supporting the hypothesis that a decline in mit

SUBMITTER: Patergnani S 

PROVIDER: S-EPMC7554583 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature