Role of CFTR protein

Question

Hello,
Can you explain in great details the mechanisms of antimicrobial immune defense in CF and the role of CFTR in these mechanisms.
Thank you for your answers

Answer

Hello,
CFTR is involved in the transport of chlroride but may have multiple other functions that are not all known. At this point, mechanismsof lung disease and abnormalities in antimicrobial defence in CF patients are not well understood and no definitive hypothesis/mechanisms have been estabilhed.
Sincerely yours

11.12.2013

11.12.13
Some helpful publications may be:

- Am J Respir Cell Mol Biol. 2013 Sep;49(3):463-70. doi: 10.1165/rcmb.2012-0502OC.
Optimal complement-mediated phagocytosis of Pseudomonas aeruginosa by monocytes is cystic fibrosis transmembrane conductance regulator-dependent.
Van de Weert-van Leeuwen PB, et al .
-J Innate Immun. 2013;5(3):195-6. doi: 10.1159/000350215. Epub 2013 Mar 23.
Neutrophils in the innate immunity conundrum of cystic fibrosis: a CFTR-related matter?
Witko-Sarsat V.
- J Pediatr (Rio J). 2013 Jan-Feb;89(1):40-7. doi: 10.1016/j.jped.2013.02.007.
The impact of cystic fibrosis on the immunologic profile of pediatric patients.
Bernardi DM, et al.
- Am J Respir Crit Care Med. 2007 Jan 15;175(2):174-83. Epub 2006 Nov 2.
A novel host defense system of airways is defective in cystic fibrosis.
Moskwa P, et al.
- Can Respir J. 2005 Jan-Feb;12(1):13-8.
Toll-like receptors 4 and 2 expression in the bronchial mucosa of patients with cystic fibrosis.
Hauber HP, et al.
- J Immunol. 2013 Jul 1;191(1):378-85. doi: 10.4049/jimmunol.1300221. Epub 2013 May 22.
Low levels of insulin-like growth factor-1 contribute to alveolar macrophage dysfunction in cystic fibrosis.
Bessich JL, et al.

D. d'Alquen