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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Detalhes bibliográficos
Main Authors: 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: Artigo
Idioma:Inglês
Publicado em: Wiley Subscription Services, Inc., A Wiley Company 2012
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Original Research Articles
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3415679/
https://ncbi.nlm.nih.gov/pubmed/21953374
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/jcp.23039
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