X-irradiation improves mdx mouse muscle as a model of myofiber loss in DMD

Journal Article · Mon Dec 31 23:00:00 EST 1990 · Muscle and Nerve; (USA)

DOI:https://doi.org/10.1002/mus.880140108· OSTI ID:6005994

Wakeford, S; Watt, D J; Partridge, T A ^[1]

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1. Charing Cross and Westminster Medical School, London (England)

The mdx mouse, although a genetic and biochemical homologue of human Duchenne muscular dystrophy (DMD), presents a comparatively mild histopathological and clinical phenotype. These differences are partially attributable to the greater efficacy of regeneration in the mdx mouse than in DMD muscle. To lessen this disparity, we have used a single dose of X-irradiation (16 Gy) to inhibit regeneration in one leg of mdx mice. The result is an almost complete block of muscle fiber regeneration leading to progressive loss of muscle fibers and their replacement by loose connective tissue. Surviving fibers are mainly peripherally nucleated and, surprisingly, of large diameter. Thus, X-irradiation converts mdx muscle to a model system in which the degenerative process can be studied in isolation from the complicating effect of myofiber regeneration. This system should be of use for testing methods of alleviating the myofiber degeneration which is common to mdx and DMD.

• OSTI ID: 6005994
• Journal Information: Muscle and Nerve; (USA), Journal Name: Muscle and Nerve; (USA) Vol. 14:1; ISSN 0148-639X; ISSN MUNED
• Country of Publication: United States
• Language: English

Related Subjects

560152* -- Radiation Effects on Animals-- Animals
63 RADIATION, THERMAL, AND OTHER ENVIRON. POLLUTANT EFFECTS ON LIVING ORGS. AND BIOL. MAT.
ANIMALS
BIOLOGICAL EFFECTS
BIOLOGICAL MODELS
BIOLOGICAL RADIATION EFFECTS
BIOLOGICAL RECOVERY
BIOLOGICAL REGENERATION
DISEASES
ELECTROMAGNETIC RADIATION
IONIZING RADIATIONS
MAMMALS
MICE
MUSCLES
NERVOUS SYSTEM DISEASES
PHENOTYPE
RADIATION EFFECTS
RADIATIONS
RECOVERY
RODENTS
VERTEBRATES
X RADIATION