Bone is functionally impaired in dystrophic mice but less so than skeletal muscle

Neuromuscul Disord. 2011 Mar;21(3):183-93. doi: 10.1016/j.nmd.2010.12.002. Epub 2011 Jan 21.

Abstract

The primary purpose of this study was to determine if tibial bone strength is compromised in dystrophic mice and if so, what geometric and material properties contribute. Results of three-point bending tests showed that tibia of mdx and dko (dystrophin- and utrophin-deficient) mice had up to 50% lower strength and stiffness compared to wild-type mice. Micro-computed tomography indicated that dystrophic tibia had reductions of 6-57% in cortical cross-sectional moment of inertia and cross-sectional area. Metaphyseal trabecular bone morphometry was also altered up to 78% in dystrophic mice. Bone-to-muscle functional ratios (i.e., three-point bending measures:muscle strength) indicated that bone strength was relatively high in 7-week-old dystrophic mice compared to muscle strength, but ratios were similar to wild-type mice by 24 months of age. Young dystrophic mice have compromised bone strength; these models may be useful for designing therapeutic regimens aimed at improving the skeleton.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Age Factors
  • Analysis of Variance
  • Animals
  • Bone Density
  • Bone Diseases / etiology*
  • Bone Diseases / genetics
  • Bone and Bones / pathology
  • Bone and Bones / physiopathology
  • Disease Models, Animal
  • Dystrophin / genetics
  • Dystrophin / metabolism*
  • Female
  • Genotype
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Inbred mdx
  • Mice, Knockout
  • Muscle Strength / physiology
  • Muscle, Skeletal / pathology*
  • Muscular Dystrophy, Duchenne / complications*
  • Muscular Dystrophy, Duchenne / genetics
  • Muscular Dystrophy, Duchenne / pathology*
  • Stress, Mechanical
  • Utrophin / deficiency

Substances

  • Dystrophin
  • Utrophin