THE mdx MOUSE SKELETAL MUSCLE MYOPATHY: I. A HISTOLOGICAL, MORPHOMETRIC AND BIOCHEMICAL INVESTIGATION

Coulton, Gary R.; Morgan, Jennifer E.; Partridge, Terence A.; Sloper, J. C.

doi:10.1111/j.1365-2990.1988.tb00866.x

Cited by 329 publications

(247 citation statements)

References 23 publications

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“…However, the extent of fibrosis in the mdx mouse does not parallel the clinical situation in that the mdx mouse has a greater regenerative capacity than DMD patients and shows substantially less replacement of muscle fibres with fibrotic and adipose tissue. 19 Therefore, it is a possibility that much higher levels of connective tissue seen in muscular dystrophy patients may hinder the electrotransfer process and that current protocols will have to be adapted further.…”

Section: Plasmid Gene Transfermentioning

confidence: 99%

High-efficiency plasmid gene transfer into dystrophic muscle

et al. 2003

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Section: Plasmid Gene Transfermentioning

confidence: 99%

High-efficiency plasmid gene transfer into dystrophic muscle

et al. 2003

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“…The animals became progressively less active, with concomitant weight loss, before premature death at -12 months of age. In contrast, rndx mice display virtually normal external appearance and normal murine life spans of 1.5-2 years (Coulton et al 1988).…”

Section: Mice Lacking Myod and Dystrophin Exhibit Increased Myopathymentioning

confidence: 99%

“…This regeneration results in the formation of additional muscle fibers, which leads to a significant level of muscle hypertrophy. Hypertrophy in rndx muscle is characterized by a 25% increase in the number of fibers, a four-fold increase in the distribution of the fiber calibers, centrally located nuclei within 70%-80% of all fibers, and a 1 .-/-fold increase in muscle mass (Anderson et al 1987;Carnwath and Shotton 1987;Coulton et al 1988; e.g., see Fig. 2, below; Table 1).…”

Section: Mice Lacking Myod and Dystrophin Exhibit Increased Myopathymentioning

confidence: 99%

“…These data suggest that MyoD has a role in the postnatal growth of skeletal muscle. The rndx phenotype is characterized by an -1.7-fold increase in the mass of individual skeletal muscles compared with wild-type mice (Anderson et al 1987;Carnwath and Shotton 1987;Coulton et al 1988; e.g., see Fig. 2 and Table 1).…”

Section: Mice Lacking Myod and Dystrophin Exhibit Increased Myopathymentioning

confidence: 99%

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MyoD is required for myogenic stem cell function in adult skeletal muscle.

Megeney¹,

Kablar²,

Garrett³

et al. 1996

Genes Dev.

643

535

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To investigate the function of MyoD in adult skeletal muscle, we interbred MyoD mutant mice with mdx mice, a model for Duchenne and Becker muscular dystrophy. Mice lacking both MyoD and dystrophin displayed a marked increase in severity of myopathy leading to premature death, suggesting a role for MyoD in muscle regeneration. Examination of MyoD mutant muscle revealed elevated numbers of myogenic cells; however, myoblasts derived from these cells displayed normal differentiation potential in vitro. Following injury, MyoD mutant muscle was severely deficient in regenerative ability, and we observed a striking reduction in the in vivo proliferation of myogenic cells during regeneration. Therefore, we propose that the failure of MyoD-deficient muscle to regenerate efficiently is not caused by a reduction in numbers of satellite cells, the stem cells of adult skeletal muscle, but results from an increased propensity for stem-cell self-renewal rather than progression through the myogenic program. The myogenic regulatory factors (MRFs] form a group of basic helix-loophelix (bHLHJ transcription factors consisting of MyoD, myogenin, Myf-5, and MRF4. The MRFs are expressed exclusively in skeletal muscle, and forcing their expression in a wide range of cultured cells induces the skeletal muscle differentiation program. Therefore, these transcription factors were postulated to have a master regulatory role in the development of the skeletal muscle lineage (for review, see Weintraub et al.

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“…Body weight of mdx mice was significantly lower than that of B10 mice at 4 weeks of age, but was similar at 6 weeks. Myofiber necrosis has been previously observed in mdx mice at 3 weeks of age, followed by myofiber regeneration (Coulton et al 1988;McGeachie et al 1993;Grounds and Torrisi 2004). Additionally, a large number of necrotic myofibers have been observed between 3 and 4 weeks of age in CT muscle (Shavlakadze et al 2004).…”

Section: Discussionmentioning

confidence: 93%

Degenerative and regenerative features of myofibers differ among skeletal muscles in a murine model of muscular dystrophy

Ikeda

Ichii

Otsuka-Kanazawa

et al. 2016

J Muscle Res Cell Motil

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Skeletal muscle myofibers constantly undergo degeneration and regeneration. Histopathological features of 6 skeletal muscles (cranial tibial [CT], gastrocnemius, quadriceps femoris, triceps brachii [TB], lumbar longissimus muscles, and costal part of the diaphragm [CPD]) were compared using C57BL/10ScSn-Dmd mdx (mdx) mice, a model for muscular dystrophy versus control, C57BL/10 mice.Body weight and skeletal muscle mass were lower in mdx mice than the control at 4 weeks of age; these results were similar at 6-30 weeks. Additionally, muscular lesions were observed in all examined skeletal muscles in mdx mice after 4 weeks, but none were noted in the controls. Immunohistochemical staining revealed numerous paired box 7-positive satellite cells surrounding the embryonic myosin heavy chain-positive regenerating myofibers, while the number of the former and staining intensity of the latter decreased as myofiber regeneration progressed. Persistent muscular lesions were observed in skeletal muscles of mdx mice between 4-14 weeks of age, and normal myofibers decreased with age. Number of muscular lesions was lowest in CPD at all ages examined, while the ratio of normal myofibers was lowest in TB at 6 weeks. In CT, TB, and CPD, Iba1-positive macrophages, the main inflammatory cells in skeletal muscle lesions, showed a significant positive correlation with the appearance of regenerating myofibers. Additionally, B220-positive B-cells showed positive and negative correlation with regenerating and regenerated myofibers, respectively. Our data suggest that degenerative and regenerative features of myofibers differ among skeletal muscles and that inflammatory cells are strongly associated with regenerative features of myofibers in mdx mice.

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THE mdx MOUSE SKELETAL MUSCLE MYOPATHY: I. A HISTOLOGICAL, MORPHOMETRIC AND BIOCHEMICAL INVESTIGATION

Cited by 329 publications

References 23 publications

High-efficiency plasmid gene transfer into dystrophic muscle

High-efficiency plasmid gene transfer into dystrophic muscle

MyoD is required for myogenic stem cell function in adult skeletal muscle.

Degenerative and regenerative features of myofibers differ among skeletal muscles in a murine model of muscular dystrophy

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