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Published on: July 3, 2020
Osteopenia in Cftr-deltaF508 mice
Josée Paradis1, Martina Wilke, Christina K Haston
1Meakins-Christie Laboratories, Department of Medicine, McGill University, Canada.
Background:
Mice with the cystic fibrosis transmembrane conductance regulator (Cftr) gene knocked out develop osteopenia. To determine whether this phenotype is present in cystic fibrosis mouse models with the DeltaF508 Cftr mutation we assessed the femora of adult FVB/N Cftr(tm1Eur) and C57BL/6 Cftr(tm1Kth) mice.
Methods:
Bone disease, relative to littermate controls, was measured using histology, densitometry and quantitative imaging.
Results:
C57BL/6 Cftr(tm1Kth) mice had shorter femurs and bones of lower volume due to thinner trabeculae, compared to wild type littermates. FVB/N Cftr(tm1Eur) mice also presented a lower bone volume which was due to significantly fewer trabeculae in this strain. Osteoblast and osteoclast numbers did not differ between CF and controls, for either of FVB/N Cftr(tm1Eur) or C57BL/6 Cftr(tm1Kth) mice. The bone architecture of FVB/N Cftr(tm1Eur) mice did not significantly differ from that of C57BL/6 Cftr(tm1Kth) mice.
Conclusions:
An osteopenic bone disease is evident in adult DeltaF508-Cftr cystic fibrosis mouse models.
Insights
Cystic fibrosis mouse models with the DeltaF508 mutation exhibit osteopenic bone disease. Bone volume is reduced due to thinner or fewer trabeculae in these models.
Area of Science:
- Bone biology
- Genetics
- Pathology
Background:
- Cystic fibrosis transmembrane conductance regulator (Cftr) gene knockout mice develop osteopenia.
- The DeltaF508 mutation is the most common cause of cystic fibrosis in humans.
Purpose of the Study:
- To investigate osteopenia in cystic fibrosis mouse models with the DeltaF508 Cftr mutation.
- To assess the bone phenotype in adult FVB/N Cftr(tm1Eur) and C57BL/6 Cftr(tm1Kth) mice.
Main Methods:
- Histology, densitometry, and quantitative imaging were used to measure bone disease.
- Femora of adult FVB/N Cftr(tm1Eur) and C57BL/6 Cftr(tm1Kth) mice were analyzed relative to littermate controls.
Main Results:
- C57BL/6 Cftr(tm1Kth) mice had shorter femurs and lower bone volume due to thinner trabeculae.
- FVB/N Cftr(tm1Eur) mice also showed lower bone volume, attributed to significantly fewer trabeculae.
- Osteoblast and osteoclast numbers did not differ between cystic fibrosis and control mice.
Conclusions:
- Adult DeltaF508-Cftr cystic fibrosis mouse models display osteopenic bone disease.
- The study confirms a link between the DeltaF508 Cftr mutation and bone loss in mice.
