Back to Search
Start Over
Severe osteopenia in CFTR-null mice
- Source :
- BONE, BONE, Elsevier, 2004, 35 (3), pp.595-603. ⟨10.1016/j.bone.2004.05.021⟩, BONE, 2004, 35 (3), pp.595-603. ⟨10.1016/j.bone.2004.05.021⟩
- Publication Year :
- 2004
-
Abstract
- Osteoporosis is a common complication in cystic fibrosis (CF) patients. In this study, we performed a histomorphometric analysis of the bones of a mouse genetic model of human CF in which both copies of the cystic fibrosis transmembrane conductance regulator (CFTR) gene are inactivated. We find that, even in the absence of obvious nutritional and therapeutic differences, the CFTR mutation is associated with severe osteopenia. Bone mineral density (BMD) of total body and of individual bones is significantly diminished. CFTR mutants display a striking significant (50%) reduction of cortical bone width and thinner trabeculae. Analysis of dynamic parameters indicates a significant reduction of bone formation and a concomitant strong increase in bone resorption. Active osteoclasts where found mostly associated with cortical bone. Our data support the concept that CF-associated osteoporosis is part of the syndromic symptoms associated with the CFTR mutation.
- Subjects :
- Pathology
Bone density
Cystic Fibrosis
Physiology
[SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology
Endocrinology, Diabetes and Metabolism
Osteoporosis
Cystic Fibrosis Transmembrane Conductance Regulator
MESH: Mice, Knockout
Cystic fibrosis
Mice
0302 clinical medicine
Bone Density
MESH: Animals
MESH: Gene Silencing
MESH: Bone Density
[SDV.BDD]Life Sciences [q-bio]/Development Biology
ComputingMilieux_MISCELLANEOUS
MESH: Cystic Fibrosis Transmembrane Conductance Regulator
Bone mineral
Mice, Knockout
0303 health sciences
biology
Cystic fibrosis transmembrane conductance regulator
medicine.anatomical_structure
MESH: Mice, Inbred CFTR
[SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]
MESH: Osteoporosis
congenital, hereditary, and neonatal diseases and abnormalities
medicine.medical_specialty
Histology
MESH: Cystic Fibrosis
Bone resorption
[SDV.BDLR.RS]Life Sciences [q-bio]/Reproductive Biology/Sexual reproduction
03 medical and health sciences
Internal medicine
medicine
Animals
Mice, Inbred CFTR
Gene Silencing
MESH: Mice
030304 developmental biology
business.industry
[SDV.BDLR]Life Sciences [q-bio]/Reproductive Biology
[SDV.BDD.MOR]Life Sciences [q-bio]/Development Biology/Morphogenesis
medicine.disease
Osteopenia
Bone Diseases, Metabolic
[SDV.BDD.EO]Life Sciences [q-bio]/Development Biology/Embryology and Organogenesis
Endocrinology
030228 respiratory system
biology.protein
Cortical bone
business
MESH: Bone Diseases, Metabolic
Subjects
Details
- ISSN :
- 87563282
- Volume :
- 35
- Issue :
- 3
- Database :
- OpenAIRE
- Journal :
- Bone
- Accession number :
- edsair.doi.dedup.....bfba92e110736170e255d75b279f4ae8