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Immune system and CF


in another answer you are writing about "hints from research that confirm a dysfunction of the immune system in CF".

What does that mean and which new approaches result from this?


[Comment by ECORN-CF team: This question refers to another question "Flu vaccine as nasal spray" (29 Oct 2012) which can be found in the Central Archive of ECORN-CF:[showitem]=2110&tx_expertadvice_pi1[search]= ]


Please let me give you some details about such an approach which are the outcome of the newer research results. One has to keep in mind, though, that the results come from basic research and that it is still very uncertain if a transfer to actual therapy concepts is possible.

As an example: What argues for a direct defect of the immune system in CF?
It could be shown that new-born CF-pigs have severe problems in avoiding a bacterial colonisation of their lungs. (Pezzuolo et al., Nature, 2012)
Back in 2006, it was reported that so called macrophages of CF-patients are not able - after ingestion of pathogens (bacteria) - to kill these germs. (Anke et al., Nature Cell Biol., 2006) This seems to be caused by a malfunctioning phagocytosis and elimination of the bacteria in the phagolysosomes. (Lamothe et al, Microbiology, 2008)

Among other authors the group of Kunzelmann could show that the channel for chloride ions Anoctamin 6, e.g., is also expressed in the macrophages (Kunzelmann et al., Europ J Physiology, 2011)
It is known that so called purinergic receptors exist in macrophages and that their stimulation is extremely important for the elimintation of bacteria which were ingested by the macrophages before. Furthermore, Kunzelmann and his group could show that the channel for chloride ions Anoctamin 6 is activated during such a purinergic stimulation. This leads to the assumption that Anoctamin 6 plays a central role for phagocytosis and the elimination of bacteria.

In CF patients the function of the channel for chloride ions Anoctamin 6 is malfunctioning due to the CFTR defect. This leads to the effect that pathogens, e.g. in the lungs of a CF patient, cannot be eliminated. It would be a promising approach to research, thus, to find chemical substances which could normalise the function of Anoctamin 6 or replace Anoctamin 6.

Best regards,
Dr. H.-G. Posselt