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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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| Autori principali: | , , , , , , , , , , , , |
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| Natura: | Artigo |
| Lingua: | Inglês |
| Pubblicazione: |
Blackwell Publishing Ltd
2009
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| Soggetti: | |
| Accesso online: | 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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