Skip to main content
Log in

Skeletal muscle pathology in X chromosome-linked muscular dystrophy (mdx) mouse

  • Original Works
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

Summary

Histological, histochemical, and morphometric analyses were performed chronologically on muscles from mutant mice with X chromosome-linked muscular dystrophy (mdx), and the findings were compared with those in nondystrophic control animals (C57BL/10ScSn). Massive grouped muscle fiber destruction, followed by complete regeneration, occurred abruptly at 20 days of age. There were no preceding changes in body weight, the number and mean diameter of fibers, and fiber type differentiation before the initial episode of muscle fiber necrosis. Muscle fiber necrosis decreased in intensity after 60 days of age. Even after repeated muscle fiber necrosis and regeneration, the most striking finding was that interstitial fibrosis and adipose tissue replacement were minimal, and there was no apparent fiber loss. Since the necrosis was probably well compensated by the active regenerative process, themdx mice developed no obvious muscle weakness and thus differed from human and other animal muscular dystrophies with the exception of the dystrophic hamster.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Ashmore CR, Doerr L (1971) Postnatal development of fiber types in normal and dystrophic skeletal muscle of the chick. Exp Neurol 30:431–446

    Google Scholar 

  • Asmundson VS, Kratzer FH, Julian LM (1966) Inherited myopathy in the chicken. Ann NY Acad Sci 138:5–58

    Google Scholar 

  • Bulfield G, Siller WG, Wight PAL, Moore K (1984) X chromosome-linked muscular dystrophy (mdx) in the mouse. Proc Natl Acad Sci USA 81:1189–1192

    Google Scholar 

  • Dangain J, Vrbova G (1984) Muscle development inmdx mutant mice. Muscle Nerve 7:700–704

    Google Scholar 

  • Dubowitz V, Brooke MH (1973) Muscle biopsy. A modern approach. Saunders, London

    Google Scholar 

  • Homburger F, Baker JR, Nixon CW, Whitney R (1962) Primary generalized polymyopathy and cardiac necrosis in an inbred line of Syrian hamsters. Med Exp 6:339–345

    Google Scholar 

  • Karpati G (1979) General discussion on hamster dystrophy. Ann NY Acad Sci 317:89–91

    Google Scholar 

  • Karpati G, Carpenter S, Prescott S (1982) Prevention of skeletal muscle fiber necrosis in hamster dystrophy. Muscle Nerve 5:369–372

    Google Scholar 

  • Mastaglia FL, Papadimitriou JM, Kakulas BA (1970) Regeneration of muscle in Duchenne muscular dystrophy: an electron microscope study. J Neurol Sci 11:425–444

    Google Scholar 

  • Michelson AM, Russel ES, Harman PJ (1955) Dystrophia muscularis: A hereditary primary myopathy in the house mouse. Proc Natl Acad Sci USA 41:1079–1084

    Google Scholar 

  • Nonaka I, Sugita H (1981) Intracytoplasmic vacuoles in αW fibers of dystrophic chicken muscle—probable early pathologic event initiates massive fiber necrosis. Acta Neuropathol (Berl) 55:173–181

    Google Scholar 

  • Nonaka I, Takagi A, Sugita H (1981) The significance of type 2C muscle fibers in Duchenne muscular dystrophy. Muscle Nerve 4:326–333

    Google Scholar 

  • Nonaka I, Nakamura H (1982) Muscle differentiation and regeneration in chicken muscular dystrophy. In: Ebashi S (ed) Muscular dystrophy, University of Tokyo Press, Tokyo, pp 63–77

    Google Scholar 

  • Wirtz P, Loermans HMTH, Peer PGM, Reintjes AGM (1983) Postnatal growth and differentiation investigation of muscle fibers in the mouse. II. A histochemical and morphometrical investigation of dystrophic muscle. J Anat 137:127–142

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Tanabe, Y., Esaki, K. & Nomura, T. Skeletal muscle pathology in X chromosome-linked muscular dystrophy (mdx) mouse. Acta Neuropathol 69, 91–95 (1986). https://doi.org/10.1007/BF00687043

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00687043

Key words

Navigation