Article
CFTR mutation is associated with bone differentiation abnormalities in cystic fibrosis.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jul 2025
Dumortier Claire, Frauenpreis Andrew, Hoarau Antony, Ryan Amy L, Gangloff Sophie C, Danopoulos Soula, Velard Frédéric, Al Alam Denise
Abstract excerpt
BACKGROUND: Cystic Fibrosis-related Bone Disease is an emerging challenge faced by 50 % of adult people with cystic fibrosis (CF). The multifactorial causes of this comorbidity remain elusive. However, congenital bone defects have been observed in animal models with CFTR mutations, suggesting its importance. The role of CFTR in bone cells development is unknown. Studies from human cells remain somewhat...
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