CFTR-deficient pigs display alterations of bone microarchitecture and composition at birth.
Animals
Animals, Newborn
Bone Density
Cancellous Bone
/ diagnostic imaging
Cortical Bone
/ diagnostic imaging
Cystic Fibrosis
/ metabolism
Cystic Fibrosis Transmembrane Conductance Regulator
/ metabolism
Disease Models, Animal
Femur
/ diagnostic imaging
Spectrum Analysis, Raman
/ methods
Swine
X-Ray Microtomography
/ methods
Bone disease
Cftr
Cortical bone
Cystic fibrosis
Femur
Pigs
Trabecular bone
Journal
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
ISSN: 1873-5010
Titre abrégé: J Cyst Fibros
Pays: Netherlands
ID NLM: 101128966
Informations de publication
Date de publication:
05 2020
05 2020
Historique:
received:
17
04
2019
revised:
07
10
2019
accepted:
22
10
2019
pubmed:
4
12
2019
medline:
14
9
2021
entrez:
3
12
2019
Statut:
ppublish
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 We compared bone parameters of CFTR The integrity of the CFTR The loss of CFTR directly alters the bone composition and metabolism of newborn pigs. Based on these findings, we speculate that bone defects in patients with CF could be a primary, rather than a secondary consequence of inflammation and infection.
Sections du résumé
BACKGROUND
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
METHODS
We compared bone parameters of CFTR
RESULTS
The integrity of the CFTR
CONCLUSIONS
The loss of CFTR directly alters the bone composition and metabolism of newborn pigs. Based on these findings, we speculate that bone defects in patients with CF could be a primary, rather than a secondary consequence of inflammation and infection.
Identifiants
pubmed: 31787573
pii: S1569-1993(19)30938-5
doi: 10.1016/j.jcf.2019.10.023
pii:
doi:
Substances chimiques
Cystic Fibrosis Transmembrane Conductance Regulator
126880-72-6
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
466-475Informations de copyright
Copyright © 2019. Published by Elsevier B.V.
Déclaration de conflit d'intérêts
Declaration of Competing Interest All authors report no conflicts of interest in this work.