X‐Irradiation improves mdx mouse muscle as a model of myofiber loss in DMD
作者:
Susan Wakeford,
Diana J. Watt,
Terence A. Partridge,
期刊:
Muscle&Nerve
(WILEY Available online 1991)
卷期:
Volume 14,
issue 1
页码: 42-50
ISSN:0148-639X
年代: 1991
DOI:10.1002/mus.880140108
出版商: Wiley Subscription Services, Inc., A Wiley Company
关键词: Duchenne muscular dystrophy;mdx DMD model;myofiber degeneration
数据来源: WILEY
摘要:
AbstractThe 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 a leviating the myofiber degeneration which is common to mdx and
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