Development of muscle pathology in canine X-linked muscular dystrophy. II. Quantitative characterization of histopathological progression during postnatal skeletal muscle development

F. Cozzi, M. Cerletti, G. C. Luvoni, R. Lombardo, P. G. Brambilla, S. Faverzani, F. Blasevich, F. Cornelio, O. Pozza, M. Mora

Research output: Contribution to journalArticle

33 Citations (Scopus)

Abstract

We have characterized the time course of muscle pathology development during the postnatal maturation of quadriceps and tibialis anterior muscle in dystrophic golden retriever dogs. We determined the percentages of degenerating, regenerating, calcium-positive, hypercontracted, albumin-positive, and C3 complement fraction-positive muscle fibers and the extent of connective tissue proliferation in animals from neonate to adult. Necrotic fibers increased from days 2 to 30, decreased at 60 days (to 0.8%) and increased in older animals to a stable level of around 2%. Hypercontracted fibers peaked at 15 days (19.1%) and declined to 3.7% in adults. Regenerating fibers were numerous at 15 and 30 days (10%), declined at 60 days to 4.7% and declined further in adults. Calcium- and albumin-positive fibers peaked at 30 days (6.5% and 13.8%, respectively) and then declined to around 3% and 5%, respectively, in older dogs. In dystrophic dogs, the extent of fibrosis was significantly greater on 15 days than in controls, but did not then increase with age. In carriers, calcium- and albumin-positive fibers always expressed dystrophin abnormally. Muscle damage occurs before completion of muscle maturation in dystrophic dogs. While necrosis and hypercontraction remain stable in adults, fiber regeneration declines to very low levels. In contrast to Duchenne muscular dystrophy, muscle fibrosis in the muscle studied does not increase with age.

Original languageEnglish
Pages (from-to)469-478
Number of pages10
JournalActa Neuropathologica
Volume101
Issue number5
Publication statusPublished - 2001

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Muscle Development
Muscular Dystrophies
Canidae
Skeletal Muscle
Pathology
Muscles
Dogs
Albumins
Calcium
Fibrosis
Dystrophin
Complement C3
Duchenne Muscular Dystrophy
Connective Tissue
Regeneration
Necrosis

Keywords

  • Canine X-linked muscular dystrophy
  • Duchenne muscular dystrophy
  • Dystrophin
  • Muscle fiber necrosis

ASJC Scopus subject areas

  • Clinical Neurology
  • Pathology and Forensic Medicine
  • Neuroscience(all)

Cite this

Development of muscle pathology in canine X-linked muscular dystrophy. II. Quantitative characterization of histopathological progression during postnatal skeletal muscle development. / Cozzi, F.; Cerletti, M.; Luvoni, G. C.; Lombardo, R.; Brambilla, P. G.; Faverzani, S.; Blasevich, F.; Cornelio, F.; Pozza, O.; Mora, M.

In: Acta Neuropathologica, Vol. 101, No. 5, 2001, p. 469-478.

Research output: Contribution to journalArticle

Cozzi, F, Cerletti, M, Luvoni, GC, Lombardo, R, Brambilla, PG, Faverzani, S, Blasevich, F, Cornelio, F, Pozza, O & Mora, M 2001, 'Development of muscle pathology in canine X-linked muscular dystrophy. II. Quantitative characterization of histopathological progression during postnatal skeletal muscle development', Acta Neuropathologica, vol. 101, no. 5, pp. 469-478.
Cozzi, F. ; Cerletti, M. ; Luvoni, G. C. ; Lombardo, R. ; Brambilla, P. G. ; Faverzani, S. ; Blasevich, F. ; Cornelio, F. ; Pozza, O. ; Mora, M. / Development of muscle pathology in canine X-linked muscular dystrophy. II. Quantitative characterization of histopathological progression during postnatal skeletal muscle development. In: Acta Neuropathologica. 2001 ; Vol. 101, No. 5. pp. 469-478.
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AU - Blasevich, F.

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