CFTR-deficient pigs display alterations of bone microarchitecture and composition at birth.
Fiche publication
Date publication
novembre 2019
Journal
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Auteurs
Membres identifiés du Cancéropôle Est :
Pr PIOT Olivier, Dr VELARD Frédéric
Tous les auteurs :
Braux J, Jourdain ML, Guillaume C, Untereiner V, Piot O, Baehr A, Klymiuk N, Winter N, Berri M, Buzoni-Gatel D, Caballero I, Guillon A, Si-Tahar M, Jacquot J, Velard F
Lien Pubmed
Résumé
The lack of cystic fibrosis transmembrane conductance regulator (CFTR) function causes cystic fibrosis (CF), predisposing to severe lung disease, reduced growth and osteopenia. Both reduced bone content and strength are increasingly recognized in infants with CF before the onset of significant lung disease, suggesting a developmental origin and a possible role in bone disease pathogenesis. The role of CFTR in bone metabolism is unclear and studies on humans are not feasible. Deletion of CFTR in pigs (CFTR pigs) displays at birth severe malformations similar to humans in the intestine, respiratory tract, pancreas, liver, and male reproductive tract.
Mots clés
Bone disease, Cftr, Cortical bone, Cystic fibrosis, Femur, Pigs, Trabecular bone
Référence
J. Cyst. Fibros.. 2019 Nov 28;: