Sparing of the extraocular muscles in mdx mice with absent or reduced utrophin expression: A life span analysis

Abby A. McDonald, Sadie L. Hebert, Linda K. McLoon

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

Abstract

Sparing of the extraocular muscles in muscular dystrophy is controversial. To address the potential role of utrophin in this sparing, mdx:utrophin+/- and mdx:utrophin-/- mice were examined for changes in myofiber size, central nucleation, and Pax7-positive and MyoD-positive cell density at intervals over their life span. Known to be spared in the mdx mouse, and contrary to previous reports, the extraocular muscles from both the mdx:utrophin+/- and mdx:utrophin-/- mice were also morphologically spared. In the mdx:utrophin+/- mice, which have a normal life span compared to the mdx:utrophin-/- mice, the myofibers were larger at 3 and 12 months than the wild type age-matched eye muscles. While there was a significant increase in central nucleation in the extraocular muscles from all mdx:utrophin+/- mice, the levels were still very low compared to age-matched limb skeletal muscles. Pax7- and MyoD-positive myogenic precursor cell populations were retained and were similar to age-matched wild type controls. These results support the hypothesis that utrophin is not involved in extraocular muscle sparing in these genotypes. In addition, it appears that these muscles retain the myogenic precursors that would allow them to maintain their regenerative capacity and normal morphology over a lifetime even in these more severe models of muscular dystrophy.

Original languageEnglish (US)
Article number3088
Pages (from-to)873-887
Number of pages15
JournalNeuromuscular Disorders
Volume25
Issue number11
DOIs
StatePublished - Nov 2015

Bibliographical note

Funding Information:
Supported by National Institutes of Health EY55137 (LKM) and EY11375 from the National Eye Institute , T32EY007133 (AAM), the 3M Science & Technology Fellowship (AAM), National Institutes of Health P30-AR0507220 , the University of Minnesota Foundation , the Minnesota Lions and Lionesses , and an unrestricted grant to the Department of Ophthalmology and Visual Neurosciences from Research to Prevent Blindness, Inc .

Keywords

  • Extraocular muscle
  • Muscular dystrophy
  • MyoD
  • Pax7
  • Satellite cell
  • Utrophin

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