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RESEARCH ARTICLE
A comparison of the bone and growth phenotype of mdx, mdx:Cmah−/− and mdx:Utrn+/− murine models with the C57BL/10 wild-type mouse
Claire L. Wood, Karla J. Suchacki, Rob van ’t Hof, Will P. Cawthorn, Scott Dillon, Volker Straub, Sze Choong Wong, Syed F. Ahmed, Colin Farquharson
Disease Models & Mechanisms 2020 13: dmm040659 doi: 10.1242/dmm.040659 Published 10 January 2020
Claire L. Wood
1Division of Developmental Biology, Roslin Institute, University of Edinburgh, Midlothian, EH25 9RG, UK
2John Walton Muscular Dystrophy Research Centre, Newcastle University and Newcastle Hospitals NHS Foundation Trust, Newcastle, NE1 3BZ, UK
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  • ORCID record for Claire L. Wood
  • For correspondence: Claire.wood@ncl.ac.uk
Karla J. Suchacki
3BHF Centre for Cardiovascular Science, University of Edinburgh, Midlothian, EH25 9RG, UK
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Rob van ’t Hof
4Institute of Ageing and Chronic Disease, University of Liverpool, Liverpool, L7 8TX, UK
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Will P. Cawthorn
3BHF Centre for Cardiovascular Science, University of Edinburgh, Midlothian, EH25 9RG, UK
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Scott Dillon
1Division of Developmental Biology, Roslin Institute, University of Edinburgh, Midlothian, EH25 9RG, UK
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Volker Straub
2John Walton Muscular Dystrophy Research Centre, Newcastle University and Newcastle Hospitals NHS Foundation Trust, Newcastle, NE1 3BZ, UK
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Sze Choong Wong
5Developmental Endocrinology Research Group, School of Medicine, University of Glasgow, Glasgow, G51 4TF, UK
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Syed F. Ahmed
5Developmental Endocrinology Research Group, School of Medicine, University of Glasgow, Glasgow, G51 4TF, UK
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Colin Farquharson
1Division of Developmental Biology, Roslin Institute, University of Edinburgh, Midlothian, EH25 9RG, UK
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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:Utrn+/−, 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. The mdx:Cmah−/− mice were lighter than WT mice at 3 weeks, but heavier at 7 weeks, and showed an increased growth rate at 5 weeks. Cortical bone fraction as assessed by micro-computed tomography was greater in both mdx and mdx:Cmah−/− mice versus WT mice at 7 weeks. Tissue mineral density was also higher in mdx:Cmah−/− mice at 3 and 7 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.

This article has an associated First Person interview with the first author of the paper.

Footnotes

  • Competing interests

    The authors declare no competing or financial interests.

  • Author contributions

    Conceptualization: C.L.W., S.C.W., S.F.A., C.F.; Methodology: C.L.W., K.J.S., R.v.t.H., W.P.C., S.D., S.F.A., C.F.; Formal analysis: C.L.W., K.J.S., R.v.t.H., W.P.C., C.F.; Investigation: C.L.W.; Resources: C.L.W.; Data curation: C.L.W.; Writing - original draft: C.L.W., W.P.C., C.F.; Writing - review & editing: C.L.W., K.J.S., R.v.t.H., W.P.C., S.D., V.S., S.C.W., S.F.A., C.F.; Supervision: V.S., S.C.W., S.F.A., C.F.; Project administration: S.F.A., C.F.; Funding acquisition: C.L.W., V.S., S.C.W., S.F.A., C.F.

  • Funding

    C.L.W. is funded by the Medical Research Council/Muscular Dystrophy UK (MDUK) (MR/N020588/1). C.F. is grateful to the Biotechnology and Biological Sciences Research Council (BBSRC) for Institute Strategic Programme Grant Funding BB/P0137321. W.P.C. is funded by the Medical Research Council (MR/M021394) and supported by a Chancellor's Fellowship from the University of Edinburgh. W.P.C. and K.J.S. are also supported by the Wellcome Trust-University of Edinburgh Institutional Strategic Support Fund.

  • Supplementary information

    Supplementary information available online at http://dmm.biologists.org/lookup/doi/10.1242/dmm.040659.supplemental

  • Received May 26, 2019.
  • Accepted November 12, 2020.
  • © 2020. Published by The Company of Biologists Ltd
http://creativecommons.org/licenses/by/4.0

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attributed.

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Keywords

  • Duchenne muscular dystrophy
  • Growth
  • Skeletal development
  • Marrow adiposity
  • Micro-CT
  • Growth plate

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RESEARCH ARTICLE
A comparison of the bone and growth phenotype of mdx, mdx:Cmah−/− and mdx:Utrn+/− murine models with the C57BL/10 wild-type mouse
Claire L. Wood, Karla J. Suchacki, Rob van ’t Hof, Will P. Cawthorn, Scott Dillon, Volker Straub, Sze Choong Wong, Syed F. Ahmed, Colin Farquharson
Disease Models & Mechanisms 2020 13: dmm040659 doi: 10.1242/dmm.040659 Published 10 January 2020
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RESEARCH ARTICLE
A comparison of the bone and growth phenotype of mdx, mdx:Cmah−/− and mdx:Utrn+/− murine models with the C57BL/10 wild-type mouse
Claire L. Wood, Karla J. Suchacki, Rob van ’t Hof, Will P. Cawthorn, Scott Dillon, Volker Straub, Sze Choong Wong, Syed F. Ahmed, Colin Farquharson
Disease Models & Mechanisms 2020 13: dmm040659 doi: 10.1242/dmm.040659 Published 10 January 2020

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