Abstract
Background and purpose: We have investigated the therapeutic effects of the selective cyclophilin inhibitor D MeAla3 EtVal4 cyclosporin (Debio 025) in myopathic Col6a1−/− mice, a model of muscular dystrophies due to defects of collagen VI. Experimental approach: We have studied calcineurin activity, T cell activation, propensity to open of the permeability transition pore in mitochondria and skeletal muscle fibres, muscle ultrastructure and apoptotic rates in Col6a1−/− mice before after treatment with Debio 025.
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