Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies

Ullrich congenital muscular dystrophy and Bethlem myopathy are skeletal muscle diseases that are due to mutations in the genes encoding collagen VI, an extracellular matrix protein forming a microfibrillar network that is particularly prominent in the endomysium of skeletal muscle. Myoblasts from pa...

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Autors principals: Merlini, Luciano, Angelin, Alessia, Tiepolo, Tania, Braghetta, Paola, Sabatelli, Patrizia, Zamparelli, Alessandra, Ferlini, Alessandra, Maraldi, Nadir M., Bonaldo, Paolo, Bernardi, Paolo
Format: Artigo
Idioma:Inglês
Publicat: National Academy of Sciences 2008
Matèries:
Biological Sciences
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC2278179/
https://ncbi.nlm.nih.gov/pubmed/18362356
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0800962105
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