Cystic Fibrosis Clinical Trial
Official title:
Study of Circulating Monocytes as Predictive Biomarker of Osteoclastogenesis in Cystic Fibrosis-related Bone Loss. Impact of CFTR Correctors.
The aim of the study is to look for risk factors of developing osteoporosis in patients with
cystic fibrosis.
Blood cells called monocytes will be obtained from a blood sample. These monocytes will make
possible to form another class of cells called osteoclasts in the laboratory. These latter
cells are responsible for bone degradation and therefore promote the increase of bone
fragility. The research will focus on the characterization of these cells to better
understand their potential (low, medium or high) to increase bone fragility in patients with
cystic fibrosis.
Finally the action of CFTR modulators on these bone cells will be tested.
The aim of the study is to characterize the profile of circulating monocytes as predictive
biomarker of osteoclastogenesis in CF related bone disease.
Investigators will evaluate the presence and regulation of CD115 (MCSF receptor) and CD265
(RANK) membrane receptors of circulating monocytes of cystic fibrosis patients.
They will also analyze the impact of the F508del CFTR mutation on the differentiation of
monocytes into osteoclasts. We will 1) Quantify the training and functional activity of
osteoclasts and, 2) Test in vitro the effects of correctors and potentiators of CFTR on the
resorption activity of differentiated osteoclasts.
The study will be explained to patients and they only will have one blood test.
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