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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Dettagli Bibliografici
Autori principali: 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
Natura: Artigo
Lingua:Inglês
Pubblicazione: National Academy of Sciences 2007
Soggetti:
Biological Sciences
Accesso online: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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