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Longitudinal pathologic study of the gastrocnemius muscle group in mdx mice

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Abstract

Muscle necrosis in mdx mice is rare before the 2nd week of life, but becomes pronounced from weeks 2 to 6. There is no agreement on what happens after this age. Using sequential microscopic histology, immunohistochemistry and histomorphometry, we studied the evolution of muscle pathology throughout the mdx life span. Between 2 weeks and 3 months, multiple necrotic groups involving variable numbers of fibres were observed in the same section. During this period, most necrotic groups comprised more than 15 fibres. From 3 to 6 months, necrosis diminished progressively, involving isolated fibres or groups of two to five fibres, and most fibres had a centrally located nucleus. From 6 months onward there was progressive variation of fibre size and deposition of fibrillary collagens. Mast cell counts increased progressively until 6 months of age. Diminishing necrosis rather than increasing regeneration may explain the differences in muscle pathology observed between human DMD and mouse mdx. Mast cells play a secondary role in pathogenesis.

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Acknowledgements

We thank Professor Salvatore Dimauro for his critical reading of the manuscript and his most valuable suggestions. This work was supported by a grant from the Fondo de Investigaciones Sanitarias (FIS 94/0968) and from the Fundació la Marató TV3 (exp. 98/3010).

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Authors and Affiliations

  1. Secció Neurologia Infantil, Hospital Universitari Vall d’Hebron, Passeig Vall d’ Hebron 119-129, 08035, Barcelona, Spain

    Manuel Roig

  2. Grup de Recerca de Malalties Neuro-Metabòliques, Hospital Universitari Vall d’Hebron, Barcelona, Spain

    Manuel Roig, Josep Roma & Arnau Fargas

  3. Unitat de Recerca Biomèdica, Hospital Universitari Vall d’Hebron, Barcelona, Spain

    Francina Munell

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  1. Manuel Roig
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  2. Josep Roma
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  4. Francina Munell
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Correspondence to Manuel Roig.

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This paper was partially presented at the meeting of the Society of European Neurological Paediatrics in Barcelona, October 1999.

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Roig, M., Roma, J., Fargas, A. et al. Longitudinal pathologic study of the gastrocnemius muscle group in mdx mice. Acta Neuropathol 107, 27–34 (2004). https://doi.org/10.1007/s00401-003-0773-3

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