Mitochondrial dysfunction in the pathogenesis of Ullrich congenital muscular dystrophy and prospective therapy with cyclosporins

Ullrich congenital muscular dystrophy is a severe genetically and clinically heterogeneous muscle disorder linked to collagen VI deficiency. The pathogenesis of the disease is unknown. To assess the potential role of mitochondrial dysfunction in the onset of muscle fiber death in this form of dystro...

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Dades bibliogràfiques
Autors principals: Angelin, Alessia, Tiepolo, Tania, Sabatelli, Patrizia, Grumati, Paolo, Bergamin, Natascha, Golfieri, Cristina, Mattioli, Elisabetta, Gualandi, Francesca, Ferlini, Alessandra, Merlini, Luciano, Maraldi, Nadir M., Bonaldo, Paolo, Bernardi, Paolo
Format: Artigo
Idioma:Inglês
Publicat: National Academy of Sciences 2007
Matèries:
Biological Sciences
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC1783427/
https://ncbi.nlm.nih.gov/pubmed/17215366
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0610270104
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