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J, Muller; Vayssière, Nathalie; Royuela, Mar; Leger, M.E.; Müller, Angela; Bacou, Francis; Pons, Françoise; Hugon, Gérald; Mornet, Dominique

doi:10.1023/a:1010305801236

Cited by 66 publications

(10 citation statements)

References 57 publications

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“…The expression of alpha-sarcoglycan ( Fig. S3 A, B ) and gamma (data not shown) was weak and scarce compared to the systematic expression in myofibers of wild- type rats [27] . Altogether, these results indicate that Dmd mdx rats are bona fide dystrophin deficient animals.…”

Section: Resultsmentioning

confidence: 82%

Characterization of Dystrophin Deficient Rats: A New Model for Duchenne Muscular Dystrophy

et al. 2014

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A few animal models of Duchenne muscular dystrophy (DMD) are available, large ones such as pigs or dogs being expensive and difficult to handle. Mdx (X-linked muscular dystrophy) mice only partially mimic the human disease, with limited chronic muscular lesions and muscle weakness. Their small size also imposes limitations on analyses. A rat model could represent a useful alternative since rats are small animals but 10 times bigger than mice and could better reflect the lesions and functional abnormalities observed in DMD patients. Two lines of Dmd mutated-rats (Dmdmdx) were generated using TALENs targeting exon 23. Muscles of animals of both lines showed undetectable levels of dystrophin by western blot and less than 5% of dystrophin positive fibers by immunohistochemistry. At 3 months, limb and diaphragm muscles from Dmdmdx rats displayed severe necrosis and regeneration. At 7 months, these muscles also showed severe fibrosis and some adipose tissue infiltration. Dmdmdx rats showed significant reduction in muscle strength and a decrease in spontaneous motor activity. Furthermore, heart morphology was indicative of dilated cardiomyopathy associated histologically with necrotic and fibrotic changes. Echocardiography showed significant concentric remodeling and alteration of diastolic function. In conclusion, Dmdmdx rats represent a new faithful small animal model of DMD.

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Section: Resultsmentioning

confidence: 82%

Characterization of Dystrophin Deficient Rats: A New Model for Duchenne Muscular Dystrophy

et al. 2014

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“…For further experiments, we used just the EDL and MAS, since they have similar fibre type compositions: EDL contains ∼ 90% IIx and IIb (Rosenblatt and Parry, 1992), and MAS is composed of ∼ 95% fast type IIx and IIb fibre types (Muller et al, 2001). We first used QPCR to compare the gene expression profiles of proliferating satellite cell-derived myoblasts (after 4 days of culture) from the EDL and MAS (Fig.…”

Section: Resultsmentioning

confidence: 99%

Muscle satellite cells are a functionally heterogeneous population in both somite-derived and branchiomeric muscles

Ono

Boldrin²,

Knopp

et al. 2010

Developmental Biology

182

222

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Skeletal muscles of body and limb are derived from somites, but most head muscles originate from cranial mesoderm. The resident stem cells of muscle are satellite cells, which have the same embryonic origin as the muscle in which they reside. Here, we analysed satellite cells with a different ontology, comparing those of the extensor digitorum longus (EDL) of the limb with satellite cells from the masseter of the head. Satellite cell-derived myoblasts from MAS and EDL muscles had distinct gene expression profiles and masseter cells usually proliferated more and differentiated later than those from EDL. When transplanted, however, masseter-derived satellite cells regenerated limb muscles as efficiently as those from EDL. Clonal analysis showed that functional properties differed markedly between satellite cells: ranging from clones that proliferated extensively and gave rise to both differentiated and self-renewed progeny, to others that divided minimally before differentiating completely. Generally, masseter-derived clones were larger and took longer to differentiate than those from EDL. This distribution in cell properties was preserved in both EDL-derived and masseter-derived satellite cells from old mice, although clones were generally less proliferative. Satellite cells, therefore, are a functionally heterogeneous population, with many occupants of the niche exhibiting stem cell characteristics in both somite-derived and branchiomeric muscles.

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“…Both zones are submitted to large strains during mastication and occlusion and bone remodeling was thus likely perturbed in mdx mice where deteriorated muscles cannot achieve the same force than in control mice. Yet, the fact that the effect of dystrophy is distributed all over the mandible is coherent with all muscles being affected, although possibly at various degrees [32]. This contrasts with the more localized shape differences caused by plastic response due to food consistency, focused on the parts of the mandible that are obviously involved in the masticatory process.…”

Section: Discussionmentioning

confidence: 99%

“…It should not have affected grinding, and accordingly neither the incisor-bearing zone nor the coronoid process are involved in this kind of phenotypic response, two zones heavily affected when the function of the incisors is modified [38]. In contrast, muscular dystrophy does not affect all muscles at the same degree [32] but all are more or less affected. Hence, all functions of the mandibles, including both chewing and grinding, are likely affected by the disease, and this would explain why phenotypic changes related to dystrophy are distributed all over the mandible.…”

Section: Discussionmentioning

confidence: 99%

Epigenetic effects on the mouse mandible: common features and discrepancies in remodeling due to muscular dystrophy and response to food consistency

2010

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BackgroundIn wild populations phenotypic differentiation of skeletal structures is influenced by many factors including epigenetic interactions and plastic response to environmental influences, possibly blurring the expression of genetic differences. In contrast, laboratory animals provide the opportunity to separate environmental from genetic effects. The mouse mandible is particularly prone to such plastic variations because bone remodeling occurs late in postnatal ontogeny, in interaction with muscular loading. In order to understand the impact of this process on mandible morphology, we investigated how change in the masticatory function affects the mandible shape, and its pattern of variation. Breeding laboratory mice on food of different consistencies mimicked a natural variation in feeding ecology, whereas mice affected by the murine analogue of the Duchenne muscular dystrophy provided a case of pathological modification of the mastication process.ResultsFood consistency as well as dystrophy caused significant shape changes in the mouse mandible. Further differences were observed between laboratory strains and between sexes within strains, muscular dystrophy causing the largest morphological change. The directions of the morphological changes due to food consistency and muscular dystrophy were discrepant, despite the fact that both are related to bone remodeling. In contrast, directions of greatest variance were comparable among most groups, and the direction of the change due to sexual dimorphism was parallel to the direction of main variance.ConclusionsBone remodeling is confirmed as an important factor driving mandible shape differences, evidenced by differences due to both the consistency of the food ingested and muscular dystrophy. However, the resulting shape change will depend on how the masticatory function is affected. Muscular dystrophy caused shape changes distributed all over the mandible, all muscles being affected although possibly to a different degree. In contrast, the chewing function was mostly affected when the mice were fed on hard vs. soft food, whereas grinding likely occurred normally; accordingly, shape change was more localized. The direction of greatest variance, however, was remarkably comparable among groups, although we found a residual variance discarding age, sex, and food differences. This suggests that whatever the context in which bone remodeling occurs, some parts of the mandible such as the angular process are more prone to remodeling during late postnatal growth.

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Cited by 66 publications

References 57 publications

Characterization of Dystrophin Deficient Rats: A New Model for Duchenne Muscular Dystrophy

Characterization of Dystrophin Deficient Rats: A New Model for Duchenne Muscular Dystrophy

Muscle satellite cells are a functionally heterogeneous population in both somite-derived and branchiomeric muscles

Epigenetic effects on the mouse mandible: common features and discrepancies in remodeling due to muscular dystrophy and response to food consistency

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