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X-IRRADIATION IMPROVES MDX MOUSE MUSCLE AS A MODEL OF MYOFIBER LOSS IN DMD
Author(s): WAKEFORD S, WATT DJ, PARTRIDGE TA
Source: MUSCLE & NERVE    Volume: 14    Issue: 1    Pages: 42-50    Published: JAN 1991  
Times Cited: 103     References: 38     
Abstract: 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.
Document Type: Article
Language: English
Addresses:
1. CHARING CROSS & WESTMINSTER MED SCH, DEPT HISTOPATHOL, FULHAM PALACERD, LONDON W6 8RF, ENGLAND
2. CHARING CROSS & WESTMINSTER MED SCH, DEPT MED, LONDON W6 8RF, ENGLAND
Publisher: JOHN WILEY & SONS INC, 605 THIRD AVE, NEW YORK, NY 10158-0012
Subject Category: Clinical Neurology; Neurosciences
IDS Number: EN805
ISSN: 0148-639X
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