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Page 1
Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration.
Nat Med. 2010 Nov;16(11):1313-20. doi: 10.1038/nm.2247. Epub 2010 Oct 31.
Nat Med. 2010.
PMID: 21037586
Oxidative stress by monoamine oxidases is causally involved in myofiber damage in muscular dystrophy.
Menazza S, Blaauw B, Tiepolo T, Toniolo L, Braghetta P, Spolaore B, Reggiani C, Di Lisa F, Bonaldo P, Canton M.
Menazza S, et al. Among authors: tiepolo t.
Hum Mol Genet. 2010 Nov 1;19(21):4207-15. doi: 10.1093/hmg/ddq339. Epub 2010 Aug 17.
Hum Mol Genet. 2010.
PMID: 20716577
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Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies.
Merlini L, Angelin A, Tiepolo T, Braghetta P, Sabatelli P, Zamparelli A, Ferlini A, Maraldi NM, Bonaldo P, Bernardi P.
Merlini L, et al. Among authors: tiepolo t.
Proc Natl Acad Sci U S A. 2008 Apr 1;105(13):5225-9. doi: 10.1073/pnas.0800962105. Epub 2008 Mar 24.
Proc Natl Acad Sci U S A. 2008.
PMID: 18362356
Free PMC article.
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Genetic ablation of cyclophilin D rescues mitochondrial defects and prevents muscle apoptosis in collagen VI myopathic mice.
Palma E, Tiepolo T, Angelin A, Sabatelli P, Maraldi NM, Basso E, Forte MA, Bernardi P, Bonaldo P.
Palma E, et al. Among authors: tiepolo t.
Hum Mol Genet. 2009 Jun 1;18(11):2024-31. doi: 10.1093/hmg/ddp126. Epub 2009 Mar 17.
Hum Mol Genet. 2009.
PMID: 19293339
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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.
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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.
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Critical evaluation of the use of cell cultures for inclusion in clinical trials of patients affected by collagen VI myopathies.
Sabatelli P, Palma E, Angelin A, Squarzoni S, Urciuolo A, Pellegrini C, Tiepolo T, Bonaldo P, Gualandi F, Merlini L, Bernardi P, Maraldi NM.
Sabatelli P, et al. Among authors: tiepolo t.
J Cell Physiol. 2012 Jul;227(7):2927-35. doi: 10.1002/jcp.23039.
J Cell Physiol. 2012.
PMID: 21953374
Free PMC article.
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