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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Detaylı Bibliyografya
Asıl Yazarlar: Merlini, Luciano, Angelin, Alessia, Tiepolo, Tania, Braghetta, Paola, Sabatelli, Patrizia, Zamparelli, Alessandra, Ferlini, Alessandra, Maraldi, Nadir M., Bonaldo, Paolo, Bernardi, Paolo
Materyal Türü: Artigo
Dil:Inglês
Baskı/Yayın Bilgisi: National Academy of Sciences 2008
Konular:
Biological Sciences
Online Erişim: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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