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...

Täydet tiedot

Tallennettuna:
Bibliografiset tiedot
Päätekijät: Sabatelli, P, Palma, E, Angelin, A, Squarzoni, S, Urciuolo, A, Pellegrini, C, Tiepolo, T, Bonaldo, P, Gualandi, F, Merlini, L, Bernardi, P, Maraldi, NM
Aineistotyyppi: Artigo
Kieli:Inglês
Julkaistu: Wiley Subscription Services, Inc., A Wiley Company 2012
Aiheet:
Original Research Articles
Linkit: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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