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Mitochondrial dysfunction in the pathogenesis of Ullrich congenital muscular dystrophy and prospective therapy with cyclosporins.
Angelin A, Tiepolo T, Sabatelli P, Grumati P, Bergamin N, Golfieri C, Mattioli E, Gualandi F, Ferlini A, Merlini L, Maraldi NM, Bonaldo P, Bernardi P. Angelin A, et al. Among authors: tiepolo t. Proc Natl Acad Sci U S A. 2007 Jan 16;104(3):991-6. doi: 10.1073/pnas.0610270104. Epub 2007 Jan 10. Proc Natl Acad Sci U S A. 2007. PMID: 17215366 Free PMC article.
The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects in Col6a1-/- myopathic mice.
Tiepolo T, Angelin A, Palma E, Sabatelli P, Merlini L, Nicolosi L, Finetti F, Braghetta P, Vuagniaux G, Dumont JM, Baldari CT, Bonaldo P, Bernardi P. Tiepolo T, et al. Br J Pharmacol. 2009 Jul;157(6):1045-52. doi: 10.1111/j.1476-5381.2009.00316.x. Epub 2009 Jun 10. Br J Pharmacol. 2009. PMID: 19519726 Free PMC article.