mdx mice show progressive weakness and muscle deterioration with age

Pastoret, Christian; Sebille, Alain

doi:10.1016/0022-510x(94)00276-t

Cited by 339 publications

(333 citation statements)

References 44 publications

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“…No classical figure of ongoing degeneration-regeneration 25,35 was observed in the muscular tissue at the time (6 days) of analysis in wild-type mouse muscles ( Figure 1C, D). The transduced fibers, which were identified by ␤-gal expression, were not centronucleated ( Figure 1C, D).…”

Section: Resultsmentioning

confidence: 97%

“…The presence of fibers of smaller diameter or irregular shape is also frequently observed in regenerated muscles. 25,35,47,48 In order to mimic an accelerated degeneration of muscle tissue, we injected notexin intramuscularly in one of the two transduced legs of immunosuppressed wild-type animals 4 weeks after the electrotransfer. Immunosuppression was continued for 1 month at which time animals were killed.…”

Section: (D-f) At Higher Magnification Asterisks Localize the Same Fmentioning

confidence: 99%

“…Except the diaphragm, which is one of the most constantly solicited skeletal muscles, they do not show extensive fibrosis. 25 Depending on their age and strain, the mice present 0.1 to 1% of revertant fibers, which express a truncated form of dystrophin. 26 The lack of dystrophin makes the mdx mouse a good model for gene complementation.…”

Section: Introductionmentioning

confidence: 99%

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Electrotransfer of naked DNA in the skeletal muscles of animal models of muscular dystrophies

Vilquin

Kennel²,

Paturneau-Jouas

et al. 2001

Gene Ther

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The electrotransfer of naked DNA has recently been adapted to the transduction of skeletal muscle fibers. We investigated the short-and long-term efficacy of this methodology in wild-type animals and in mouse models of congenital muscular dystrophy (dy/dy, dy 2J /dy 2J ), or Duchenne muscular dystrophy (mdx/mdx). Using a reporter construct, the short-term efficacy of fiber transduction reached 40% and was similar in wild-type, dy/dy and dy 2J /dy 2J animals, indicating that ongoing muscle fibrosis was not a major obstacle to the electrotransfer-mediated gene transfer. Although the complete rejection of transduced fibers was observed within 3 weeks in the absence of immunosuppression, the persistency was prolonged over 10 weeks when transient or continuous immunosuppressive regimens were used. Using

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Section: Resultsmentioning

confidence: 97%

Section: (D-f) At Higher Magnification Asterisks Localize the Same Fmentioning

confidence: 99%

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Electrotransfer of naked DNA in the skeletal muscles of animal models of muscular dystrophies

Vilquin

Kennel²,

Paturneau-Jouas

et al. 2001

Gene Ther

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“…It is known that at this specific age the muscle starts to present the first signs of the disease, distinguished by an intense degeneration. 4 Consequently, the regenerative process is activated, including the activation and proliferation of new cells, that here we see represented by the rise in the expression of cell-cycle genes. Thus, further studies of the genes implicated on these functions will help to understand the milder phenotype of this mouse, revealing possible therapeutic markers.…”

Section: Characterization Of Each Dystrophic Strainmentioning

confidence: 85%

“…3 The muscles of these mice show many features of a dystrophic muscle, including degeneration and fibrosis; nevertheless, differently from DMD patients, they have a significant muscle regeneration and an almost normal phenotype; thus, Dmd mdx is good for genetic and biochemical studies, but not for clinical trials. 4 Congenital muscular dystrophies (CMD) compose a subgroup of MD, including forms caused by glycosylation defects in the α-dystroglycan protein, a member of the dystrophin-glycoprotein complex (DGC). 5 Glycosylation of α-dystroglycan is crucial for its binding to laminin in the extracellular matrix (ECM), and it is a process performed by a series of glycosyltransferase enzymes, including LARGE.…”

Section: Introductionmentioning

confidence: 99%

Comparative transcriptome analysis of muscular dystrophy models Largemyd, Dmdmdx/Largemyd and Dmdmdx: what makes them different?

2016

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Muscular dystrophies (MD) are a clinically and genetically heterogeneous group of Mendelian diseases. The underlying pathophysiology and phenotypic variability in each form are much more complex, suggesting the involvement of many other genes. Thus, here we studied the whole genome expression profile in muscles from three mice models for MD, at different time points: Dmd mdx (mutation in dystrophin gene), Large myd − / − (mutation in Large) and Dmd mdx /Large myd − / − (both mutations). The identification of altered biological functions can contribute to understand diseases and to find prognostic biomarkers and points for therapeutic intervention. We identified a substantial number of differentially expressed genes (DEGs) in each model, reflecting diseases' complexity. The main biological process affected in the three strains was immune system, accounting for the majority of enriched functional categories, followed by degeneration/regeneration and extracellular matrix remodeling processes. The most notable differences were in 21-day-old Dmd mdx , with a high proportion of DEGs related to its regenerative capacity. A higher number of positive embryonic myosin heavy chain (eMyHC) fibers confirmed this. The new Dmd mdx /Large myd − / − model did not show a highly different transcriptome from the parental lineages, with a profile closer to Large myd − / − , but not bearing the same regenerative potential as Dmd mdx . This is the first report about transcriptome profile of a mouse model for congenital MD and Dmd mdx /Large myd . By comparing the studied profiles, we conclude that alterations in biological functions due to the dystrophic process are very similar, and that the intense regeneration in Dmd mdx involves a large number of activated genes, not differentially expressed in the other two strains.

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Eye muscle sparing by the muscular dystrophies: Lessons to be learned?

Andrade

Porter

Kaminski

2000

Microsc. Res. Tech.

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The devastating consequences of the various muscular dystrophies are even more obvious when a muscle or muscle group is spared. The study of the exceptional cell or tissue responses may prove to be of considerable value in the analysis of disease mechanisms. The small muscles responsible for eye movements, the extraocular muscles, have functional and morphological characteristics that set them aside from other skeletal muscles. Notably, these muscles are clinically unaffected in Duchenne/Becker, limb‐girdle, and congenital muscular dystrophies, pathologies due to a broken mechanical or signaling linkage between the cytoskeleton and the extracellular matrix. Uncovering the strategies used by the extraocular muscles to “naturally” protect themselves in these diseases should contribute to knowledge of both pathogenesis and treatment. We propose that careful investigation of the cellular determinants of extraocular muscle‐specific properties may provide insights into how these muscles avoid or adapt to the cascade of events leading to myofiber degeneration in the muscular dystrophies. Microsc. Res. Tech. 48:192–203, 2000. © 2000 Wiley‐Liss, Inc.

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mdx mice show progressive weakness and muscle deterioration with age

Cited by 339 publications

References 44 publications

Electrotransfer of naked DNA in the skeletal muscles of animal models of muscular dystrophies

Electrotransfer of naked DNA in the skeletal muscles of animal models of muscular dystrophies

Comparative transcriptome analysis of muscular dystrophy models Largemyd, Dmdmdx/Largemyd and Dmdmdx: what makes them different?

Eye muscle sparing by the muscular dystrophies: Lessons to be learned?

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