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Critical Evaluation of the Use of Cell Cultures for Inclusion in Clinical Trials of Patients Affected by Collagen VI Myopathies

Collagen VI myopathies (Ullrich congenital muscular dystrophy (UCMD), Bethlem myopathy (BM), and myosclerosis myopathy) share a common pathogenesis, that is, mitochondrial dysfunction due to deregulation of the permeability transition pore (PTP). This effect was first identified in the Col6a1(−/−) m...

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Detalles Bibliográficos
Autores principales:	Sabatelli, P, Palma, E, Angelin, A, Squarzoni, S, Urciuolo, A, Pellegrini, C, Tiepolo, T, Bonaldo, P, Gualandi, F, Merlini, L, Bernardi, P, Maraldi, NM
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Wiley Subscription Services, Inc., A Wiley Company 2012
Materias:	Original Research Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3415679/ https://www.ncbi.nlm.nih.gov/pubmed/21953374 http://dx.doi.org/10.1002/jcp.23039

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