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Items: 1 to 20 of 102

1.

Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration.

Grumati P, Coletto L, Sabatelli P, Cescon M, Angelin A, Bertaggia E, Blaauw B, Urciuolo A, Tiepolo T, Merlini L, Maraldi NM, Bernardi P, Sandri M, Bonaldo P.

Nat Med. 2010 Nov;16(11):1313-20. doi: 10.1038/nm.2247.

PMID:
21037586
2.

Autophagy induction rescues muscular dystrophy.

Grumati P, Coletto L, Sandri M, Bonaldo P.

Autophagy. 2011 Apr;7(4):426-8. doi: 10.4161/auto.7.4.14392.

PMID:
21543891
3.

Autophagy thwarts muscle disease.

Tolkovsky AM.

Nat Med. 2010 Nov;16(11):1188-90. doi: 10.1038/nm1110-1188. No abstract available.

PMID:
21052066
4.

Physical exercise stimulates autophagy in normal skeletal muscles but is detrimental for collagen VI-deficient muscles.

Grumati P, Coletto L, Schiavinato A, Castagnaro S, Bertaggia E, Sandri M, Bonaldo P.

Autophagy. 2011 Dec;7(12):1415-23.

5.

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.

Hum Mol Genet. 2009 Jun 1;18(11):2024-31. doi: 10.1093/hmg/ddp126.

PMID:
19293339
6.

Reactivation of autophagy by spermidine ameliorates the myopathic defects of collagen VI-null mice.

Chrisam M, Pirozzi M, Castagnaro S, Blaauw B, Polishchuck R, Cecconi F, Grumati P, Bonaldo P.

Autophagy. 2015;11(12):2142-52. doi: 10.1080/15548627.2015.1108508.

7.

Mitochondrial dysfunction and defective autophagy in the pathogenesis of collagen VI muscular dystrophies.

Bernardi P, Bonaldo P.

Cold Spring Harb Perspect Biol. 2013 May 1;5(5):a011387. doi: 10.1101/cshperspect.a011387. Review.

8.

Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency.

Irwin WA, Bergamin N, Sabatelli P, Reggiani C, Megighian A, Merlini L, Braghetta P, Columbaro M, Volpin D, Bressan GM, Bernardi P, Bonaldo P.

Nat Genet. 2003 Dec;35(4):367-71.

PMID:
14625552
9.

Cyclosporine A treatment for Ullrich congenital muscular dystrophy: a cellular study of mitochondrial dysfunction and its rescue.

Hicks D, Lampe AK, Laval SH, Allamand V, Jimenez-Mallebrera C, Walter MC, Muntoni F, Quijano-Roy S, Richard P, Straub V, Lochmüller H, Bushby KM.

Brain. 2009 Jan;132(Pt 1):147-55. doi: 10.1093/brain/awn289.

PMID:
19015158
10.

Novel mutations in collagen VI genes: expansion of the Bethlem myopathy phenotype.

Scacheri PC, Gillanders EM, Subramony SH, Vedanarayanan V, Crowe CA, Thakore N, Bingler M, Hoffman EP.

Neurology. 2002 Feb 26;58(4):593-602.

PMID:
11865138
11.

Muscle Weakness and Fibrosis Due to Cell Autonomous and Non-cell Autonomous Events in Collagen VI Deficient Congenital Muscular Dystrophy.

Noguchi S, Ogawa M, Malicdan MC, Nonaka I, Nishino I.

EBioMedicine. 2017 Feb;15:193-202. doi: 10.1016/j.ebiom.2016.12.011.

12.

Zebrafish models of collagen VI-related myopathies.

Telfer WR, Busta AS, Bonnemann CG, Feldman EL, Dowling JJ.

Hum Mol Genet. 2010 Jun 15;19(12):2433-44. doi: 10.1093/hmg/ddq126.

13.

Muscle interstitial fibroblasts are the main source of collagen VI synthesis in skeletal muscle: implications for congenital muscular dystrophy types Ullrich and Bethlem.

Zou Y, Zhang RZ, Sabatelli P, Chu ML, Bönnemann CG.

J Neuropathol Exp Neurol. 2008 Feb;67(2):144-54. doi: 10.1097/nen.0b013e3181634ef7.

PMID:
18219255
14.

Deep RNA profiling identified CLOCK and molecular clock genes as pathophysiological signatures in collagen VI myopathy.

Scotton C, Bovolenta M, Schwartz E, Falzarano MS, Martoni E, Passarelli C, Armaroli A, Osman H, Rodolico C, Messina S, Pegoraro E, D'Amico A, Bertini E, Gualandi F, Neri M, Selvatici R, Boffi P, Maioli MA, Lochmüller H, Straub V, Bushby K, Castrignanò T, Pesole G, Sabatelli P, Merlini L, Braghetta P, Bonaldo P, Bernardi P, Foley R, Cirak S, Zaharieva I, Muntoni F, Capitanio D, Gelfi C, Kotelnikova E, Yuryev A, Lebowitz M, Zhang X, Hodge BA, Esser KA, Ferlini A.

J Cell Sci. 2016 Apr 15;129(8):1671-84. doi: 10.1242/jcs.175927.

15.

Autophagy activation in COL6 myopathic patients by a low-protein-diet pilot trial.

Castagnaro S, Pellegrini C, Pellegrini M, Chrisam M, Sabatelli P, Toni S, Grumati P, Ripamonti C, Pratelli L, Maraldi NM, Cocchi D, Righi V, Faldini C, Sandri M, Bonaldo P, Merlini L.

Autophagy. 2016 Dec;12(12):2484-2495.

16.

Defective collagen VI α6 chain expression in the skeletal muscle of patients with collagen VI-related myopathies.

Tagliavini F, Pellegrini C, Sardone F, Squarzoni S, Paulsson M, Wagener R, Gualandi F, Trabanelli C, Ferlini A, Merlini L, Santi S, Maraldi NM, Faldini C, Sabatelli P.

Biochim Biophys Acta. 2014 Sep;1842(9):1604-12. doi: 10.1016/j.bbadis.2014.05.033.

17.

Muscle magnetic resonance imaging in patients with congenital muscular dystrophy and Ullrich phenotype.

Mercuri E, Cini C, Pichiecchio A, Allsop J, Counsell S, Zolkipli Z, Messina S, Kinali M, Brown SC, Jimenez C, Brockington M, Yuva Y, Sewry CA, Muntoni F.

Neuromuscul Disord. 2003 Sep;13(7-8):554-8.

PMID:
12921792
18.

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.

Br J Pharmacol. 2009 Jul;157(6):1045-52. doi: 10.1111/j.1476-5381.2009.00316.x.

19.

Muscle proteomics reveals novel insights into the pathophysiological mechanisms of collagen VI myopathies.

De Palma S, Capitanio D, Vasso M, Braghetta P, Scotton C, Bonaldo P, Lochmüller H, Muntoni F, Ferlini A, Gelfi C.

J Proteome Res. 2014 Nov 7;13(11):5022-30. doi: 10.1021/pr500675e.

PMID:
25211533
20.

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.

J Cell Physiol. 2012 Jul;227(7):2927-35. doi: 10.1002/jcp.23039.

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