Regeneration in Duchenne Muscular Dystrophy: A Histological and Histochemical Study

Mastaglia, Frank; Kakulas, B. A.

doi:10.1093/brain/92.4.809

Cited by 57 publications

(14 citation statements)

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“…Despite the fact that branched myofibers are observed in several muscular dystrophies in human, dog, cat and mouse [3-11], myofiber branching has been studied predominantly studied in mdx mice, a mouse model of Duchenne muscular dystrophy (DMD) [10,12-15]. DMD is a genetic disease affecting about 1 in every 3,500 boys [16].…”

Section: Introductionmentioning

confidence: 99%

Incidence and severity of myofiber branching with regeneration and aging

Pichavant

Pavlath

2014

Skeletal Muscle

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BackgroundMyofibers with an abnormal branching cytoarchitecture are commonly found in muscular dystrophy and in regenerated or aged nondystrophic muscles. Such branched myofibers from dystrophic mice are more susceptible to damage than unbranched myofibers in vitro, suggesting that muscles containing a high percentage of these myofibers are more prone to injury. Little is known about the regulation of myofiber branching.MethodsTo gain insights into the formation and fate of branched myofibers, we performed in-depth analyses of single myofibers isolated from dystrophic and nondystrophic (myotoxin-injured or aged) mouse muscles. The proportion of branched myofibers, the number of branches per myofiber and the morphology of the branches were assessed.ResultsAged dystrophic mice exhibited the most severe myofiber branching as defined by the incidence of branched myofibers and the number of branches per myofiber, followed by myotoxin-injured, wild-type muscles and then aged wild-type muscles. In addition, the morphology of the branched myofibers differed among the various models. In response to either induced or ongoing muscle degeneration, branching was restricted to regenerated myofibers containing central nuclei. In myotoxin-injured muscles, the amount of branched myofibers remained stable over time.ConclusionWe suggest that myofiber branching is a consequence of myofiber remodeling during muscle regeneration. Our present study lays valuable groundwork for identifying the molecular pathways leading to myofiber branching in dystrophy, trauma and aging. Decreasing myofiber branching in dystrophic patients may improve muscle resistance to mechanical stress.

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Section: Introductionmentioning

confidence: 99%

Incidence and severity of myofiber branching with regeneration and aging

Pichavant

Pavlath

2014

Skeletal Muscle

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“…The mechanism of histological progression remains unclear. Previous studies suggest that there is a progressive loss of regenerating capacity in muscle fibres 1. Interstitial fibrosis may be attributed to a loss of regenerating capacity in satellite cells, which is caused by a reduced blood supply to individual muscle fibres 2, 3.…”

Section: Introductionmentioning

confidence: 99%

Foot contact surface effect to the metatarsals loading character during inversion landing

Ren

Ruan³

et al. 2010

Int. J. Numer. Meth. Biomed. Engng.

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SUMMARYThe present study reports the development of a detailed three-dimensional (3D) finite element (FE) foot model for investigating the effect of the material properties and thicknesses of the landing mat on stress distribution and concentration point within the metatarsals during landing at an inversion position. Foam material mat insert between the foot and ground with different thicknesses had been systematic studied. The predicted plantar pressure distribution showed a good agreement with the experimental data of controlled biomechanical tests. Results showed that mat insert with optimal properties and thickness could reduce the peak plantar pressure by about 36% comparing to the barefoot-solid ground condition. The fifth metatarsal was the most vulnerable part during the inversion landing, and the peak stress point was located near the proximal part. Material sensitivity study showed that the metatarsals' stress level was affected by the material property of the mat and its thickness. Meanwhile, the influence of thickness for soft material was more effective than that for the hard material.

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“…In the early stages of DMD, necrotic loss of skeletal muscle is compensated for by proliferation and differentiation of satellite cells, a resident population of muscle progenitors (140). This is supported by histological evidence showing signs of muscle regeneration, including centrally nucleated fibers and variable muscle fiber size, in DMD patients (98). Satellite cell regeneration of skeletal muscle compensates for necrotic muscle death in DMD for some time but eventually the proliferative capacity of these cells wanes, contributing to progressive muscle wasting (83,179).…”

Section: Skeletal Muscle Dysfunction and Exercise Intolerancementioning

confidence: 83%

Exercise and Muscular Dystrophy: Implications and Analysis of Effects on Musculoskeletal and Cardiovascular Systems

Barnabei

Martindale

Townsend

et al. 2011

Comprehensive Physiology

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The muscular dystrophies are a heterogeneous collection of progressive, inherited diseases of muscle weakness and degeneration. Although these diseases can vary widely in their etiology and presentation, nearly all muscular dystrophies cause exercise intolerance to some degree. Here, we focus on Duchenne muscular dystrophy (DMD), the most common form of muscular dystrophy, as a paradigm for the effects of muscle disease on exercise capacity. First described in the mid-1800s, DMD is a rapidly progressive and lethal muscular dystrophy caused by mutations in the dystrophin gene. Dystrophin is a membrane-associated cytoskeletal protein, the loss of which causes numerous cellular defects including mechanical instability of the sarcolemma, increased influx of extracellular calcium, and cell signaling defects. Here, we discuss the physiological basis for exercise intolerance in DMD, focusing on the molecular and cellular defects caused by loss of dystrophin and how these manifest as organ-level dysfunction and reduced exercise capacity. The main focus of this article is the defects present in dystrophin-deficient striated muscle. However, discussion regarding the effects of dystrophin loss on other tissues, including vascular smooth muscle is also included. Collectively, the goal of this article is to summarize the current state of knowledge regarding the mechanistic basis for exercise intolerance in DMD, which may serve as an archetype for other muscular dystrophies and diseases of muscle wasting.

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Regeneration in Duchenne Muscular Dystrophy: A Histological and Histochemical Study

Cited by 57 publications

References 0 publications

Incidence and severity of myofiber branching with regeneration and aging

Incidence and severity of myofiber branching with regeneration and aging

Foot contact surface effect to the metatarsals loading character during inversion landing

Exercise and Muscular Dystrophy: Implications and Analysis of Effects on Musculoskeletal and Cardiovascular Systems

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