The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects in Col6a1(−/−) myopathic mice

BACKGROUND AND PURPOSE: We have investigated the therapeutic effects of the selective cyclophilin inhibitor D-MeAla(3)-EtVal(4)-cyclosporin (Debio 025) in myopathic Col6a1(−/−) mice, a model of muscular dystrophies due to defects of collagen VI. EXPERIMENTAL APPROACH: We studied calcineurin activity...

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Main Authors: Tiepolo, T, Angelin, A, Palma, E, Sabatelli, P, Merlini, L, Nicolosi, L, Finetti, F, Braghetta, P, Vuagniaux, G, Dumont, J-M, Baldari, CT, Bonaldo, P, Bernardi, P
Formato: Artigo
Idioma:Inglês
Publicado: Blackwell Publishing Ltd 2009
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Research Papers
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC2737663/
https://ncbi.nlm.nih.gov/pubmed/19519726
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1111/j.1476-5381.2009.00316.x
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