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 language | English |
|---|---|
| Pages (from-to) | 469-478 |
| Number of pages | 10 |
| Journal | Acta Neuropathologica |
| Volume | 101 |
| Issue number | 5 |
| Publication status | Published - 2001 |
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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 journal › Article
}
TY - JOUR
T1 - Development of muscle pathology in canine X-linked muscular dystrophy. II. Quantitative characterization of histopathological progression during postnatal skeletal muscle development
AU - Cozzi, F.
AU - Cerletti, M.
AU - Luvoni, G. C.
AU - Lombardo, R.
AU - Brambilla, P. G.
AU - Faverzani, S.
AU - Blasevich, F.
AU - Cornelio, F.
AU - Pozza, O.
AU - Mora, M.
PY - 2001
Y1 - 2001
N2 - 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.
AB - 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.
KW - Canine X-linked muscular dystrophy
KW - Duchenne muscular dystrophy
KW - Dystrophin
KW - Muscle fiber necrosis
UR - http://www.scopus.com/inward/record.url?scp=0034957477&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0034957477&partnerID=8YFLogxK
M3 - Article
C2 - 11484818
AN - SCOPUS:0034957477
VL - 101
SP - 469
EP - 478
JO - Acta Neuropathologica
JF - Acta Neuropathologica
SN - 0001-6322
IS - 5
ER -