Current concepts of immune dysregulation in cystic fibrosis.
Int J Biochem Cell Biol
; 52: 108-12, 2014 Jul.
Article
en En
| MEDLINE
| ID: mdl-24495876
ABSTRACT
Cystic fibrosis (CF) lung disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene and is characterized by a perpetuated feedback loop of bacterial infection and inflammation. Both intrinsic (CFTR-dependent) and extrinsic (CFTR-independent) mechanisms contribute to the inflammatory phenotype of CF lung disease. Innate immune cells, initially recruited to combat bacterial pathogens, are acting in a dysregulated and non-resolving fashion in CF airways and cause harm to the host by releasing proteases and oxidants. Targeting harmful immune pathways, while preserving protective ones, remains the challenge for the future. This review highlights current concepts of innate immune dysregulation in CF lung disease.
Palabras clave
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Fibrosis Quística
Límite:
Animals
/
Humans
Idioma:
En
Revista:
Int J Biochem Cell Biol
Asunto de la revista:
BIOQUIMICA
Año:
2014
Tipo del documento:
Article
País de afiliación:
Alemania