Projects per year
Description
MicroCT of bones from DMD mouse models.
## Access ##
This dataset is held in the Edinburgh DataVault, directly accessible only to authorised University of Edinburgh users. External users may request access to a copy of the data by contacting the Principal Investigator, Contact Person or Data Manager named on this page. University of Edinburgh users who wish to have direct access should consult the information about retrieving data from the DataVault at: http://www.ed.ac.uk/is/research-support/datavault .
## Access ##
This dataset is held in the Edinburgh DataVault, directly accessible only to authorised University of Edinburgh users. External users may request access to a copy of the data by contacting the Principal Investigator, Contact Person or Data Manager named on this page. University of Edinburgh users who wish to have direct access should consult the information about retrieving data from the DataVault at: http://www.ed.ac.uk/is/research-support/datavault .
Abstract
The muscular dystrophy x-linked (mdx) mouse is commonly used as a mouse model of Duchenne muscular dystrophy (DMD). Its phenotype is, however, mild and other mouse models have been explored. The mdx:cmah mouse carries a human-like mutation in the Cmah gene and has a severe muscle phenotype, but its growth and bone development are unknown. In this study we compared male mdx, mdx:utr+/-, mdx:cmah-/- and wild-type (WT) mice at 3, 5 and 7 weeks of age to determine the suitability of the mdx:cmah-/- mouse as a model for assessing growth and skeletal development in DMD.
Mdx:cmah mice were lighter than WT mice at 3, but heavier at 7 weeks (p=0.02) and showed an increased growth rate at 5 weeks (p=0.007). Cortical bone fraction as assessed by micro-computed tomography was greater in both mdx and mdx:cmah mice v WT mice at 7 weeks (p<0.05).Tissue mineral density was also higher in mdx:cmah mice at 3 (p<0.05) and 7 (p<0.01) weeks. Gene profiling of mdx:cmah bone identified increased expression of Igf1, Igf1r and Vegfa. Both the mdx and mdx:cmah mice showed an increased proportion of regulated bone marrow adipose tissue (BMAT) but a reduction in constitutive BMAT.
The mdx:cmah mice show evidence of catch-up growth and more rapid bone development. This pattern does not mimic the typical DMD growth trajectory and therefore the utility of the mdx:cmah mouse for studying growth and skeletal development in DMD is limited. Further studies of this model may however shed light on the phenomenon of catch-up growth.
Mdx:cmah mice were lighter than WT mice at 3, but heavier at 7 weeks (p=0.02) and showed an increased growth rate at 5 weeks (p=0.007). Cortical bone fraction as assessed by micro-computed tomography was greater in both mdx and mdx:cmah mice v WT mice at 7 weeks (p<0.05).Tissue mineral density was also higher in mdx:cmah mice at 3 (p<0.05) and 7 (p<0.01) weeks. Gene profiling of mdx:cmah bone identified increased expression of Igf1, Igf1r and Vegfa. Both the mdx and mdx:cmah mice showed an increased proportion of regulated bone marrow adipose tissue (BMAT) but a reduction in constitutive BMAT.
The mdx:cmah mice show evidence of catch-up growth and more rapid bone development. This pattern does not mimic the typical DMD growth trajectory and therefore the utility of the mdx:cmah mouse for studying growth and skeletal development in DMD is limited. Further studies of this model may however shed light on the phenomenon of catch-up growth.
| Date made available | 2019 |
|---|---|
| Publisher | Edinburgh DataVault |
Projects
- 1 Finished
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Characterisation of skeletal development and the use of anabolic agents in murine models of Duchenne Muscular Dystrophy
Wood, C.
11/04/16 → 19/07/19
Project: Research