Role of innate immunity and systemic inflammation in cystic fibrosis disease progression
Anand Kumar Purushothaman, Everette Jacob Remington Nelson
Abstract
, loss of CFTR function could affect various basic cellular processes, such as cellular homeostasis, lysosome acidification, and redox balance. CFTR aids in endotoxin tolerance by regulating Toll-like receptor-mediated signaling resulting in uncontrolled activation of innate immune cells. Although leukocytes of CF patients are hyperactivated, they exhibit compromised phagosome activity thus favouring the orchestration of sepsis from defective pathogen clearance. This review will emphasize the importance of innate immunity and systemic inflammatory response in the development of CF and other CFTR-associated pathologies.
Topics & Concepts
Cystic fibrosisCystic fibrosis transmembrane conductance regulatorInnate immune systemImmunologyInflammationImmune systemImmunityBiologyFibrosisAcquired immune systemCell biologyMedicinePathologyGeneticsCystic Fibrosis Research AdvancesNeonatal Respiratory Health ResearchRespiratory Support and Mechanisms