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Distinct Fiber Type Signature in Mouse Muscles Expressing a Mutant Lamin A Responsible for Congenital Muscular Dystrophy in a Patient
Specific mutations in LMNA, which encodes nuclear intermediate filament proteins lamins A/C, affect skeletal muscle tissues. Early-onset LMNA myopathies reveal different alterations of muscle fibers, including fiber type disproportion or prominent dystrophic and/or inflammatory changes. Recently, we...
Autores principales: | Barateau, Alice, Vadrot, Nathalie, Agbulut, Onnik, Vicart, Patrick, Batonnet-Pichon, Sabrina, Buendia, Brigitte |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5492014/ https://www.ncbi.nlm.nih.gov/pubmed/28441765 http://dx.doi.org/10.3390/cells6020010 |
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