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Items: 1 to 20 of 110

1.

Improving clinical trial design for Duchenne muscular dystrophy.

Merlini L, Sabatelli P.

BMC Neurol. 2015 Aug 26;15:153. doi: 10.1186/s12883-015-0408-z.

2.

Detecting collagen VI in Bethlem myopathy.

Sabatelli P, Gualandi F, Bonaldo P, Merlini L.

J Biol Chem. 2015 Mar 20;290(12):8011. doi: 10.1074/jbc.L115.639088. No abstract available.

PMID:
25795730
3.

Melanocytes from Patients Affected by Ullrich Congenital Muscular Dystrophy and Bethlem Myopathy have Dysfunctional Mitochondria That Can be Rescued with Cyclophilin Inhibitors.

Zulian A, Tagliavini F, Rizzo E, Pellegrini C, Sardone F, Zini N, Maraldi NM, Santi S, Faldini C, Merlini L, Petronilli V, Bernardi P, Sabatelli P.

Front Aging Neurosci. 2014 Nov 20;6:324. doi: 10.3389/fnagi.2014.00324. eCollection 2014.

4.

Aggresome-Autophagy Involvement in a Sarcopenic Patient with Rigid Spine Syndrome and a p.C150R Mutation in FHL1 Gene.

Sabatelli P, Castagnaro S, Tagliavini F, Chrisam M, Sardone F, Demay L, Richard P, Santi S, Maraldi NM, Merlini L, Sandri M, Bonaldo P.

Front Aging Neurosci. 2014 Aug 19;6:215. doi: 10.3389/fnagi.2014.00215. eCollection 2014.

5.

Monoamine oxidase inhibition prevents mitochondrial dysfunction and apoptosis in myoblasts from patients with collagen VI myopathies.

Sorato E, Menazza S, Zulian A, Sabatelli P, Gualandi F, Merlini L, Bonaldo P, Canton M, Bernardi P, Di Lisa F.

Free Radic Biol Med. 2014 Oct;75:40-7. doi: 10.1016/j.freeradbiomed.2014.07.006. Epub 2014 Jul 10.

6.

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. Epub 2014 Jun 5.

7.

NIM811, a cyclophilin inhibitor without immunosuppressive activity, is beneficial in collagen VI congenital muscular dystrophy models.

Zulian A, Rizzo E, Schiavone M, Palma E, Tagliavini F, Blaauw B, Merlini L, Maraldi NM, Sabatelli P, Braghetta P, Bonaldo P, Argenton F, Bernardi P.

Hum Mol Genet. 2014 Oct 15;23(20):5353-63. doi: 10.1093/hmg/ddu254. Epub 2014 May 22.

8.

Ultrastructural changes in muscle cells of patients with collagen VI-related myopathies.

Tagliavini F, Sardone F, Squarzoni S, Maraldi NM, Merlini L, Faldini C, Sabatelli P.

Muscles Ligaments Tendons J. 2014 Feb 24;3(4):281-6. eCollection 2013 Oct.

9.

Biodistribution and molecular studies on orally administered nanoparticle-AON complexes encapsulated with alginate aiming at inducing dystrophin rescue in mdx mice.

Falzarano MS, Passarelli C, Bassi E, Fabris M, Perrone D, Sabatelli P, Maraldi NM, Donà S, Selvatici R, Bonaldo P, Sparnacci K, Laus M, Braghetta P, Rimessi P, Ferlini A.

Biomed Res Int. 2013;2013:527418. doi: 10.1155/2013/527418. Epub 2013 Dec 12.

10.

Effect of mechanical strain on the collagen VI pericellular matrix in anterior cruciate ligament fibroblasts.

Sardone F, Traina F, Tagliavini F, Pellegrini C, Merlini L, Squarzoni S, Santi S, Neri S, Faldini C, Maraldi N, Sabatelli P.

J Cell Physiol. 2014 Jul;229(7):878-86. doi: 10.1002/jcp.24518.

PMID:
24356950
11.

EMILIN1/α9β1 integrin interaction is crucial in lymphatic valve formation and maintenance.

Danussi C, Del Bel Belluz L, Pivetta E, Modica TM, Muro A, Wassermann B, Doliana R, Sabatelli P, Colombatti A, Spessotto P.

Mol Cell Biol. 2013 Nov;33(22):4381-94. doi: 10.1128/MCB.00872-13. Epub 2013 Sep 9.

12.

Characterization of a rare case of Ullrich congenital muscular dystrophy due to truncating mutations within the COL6A1 gene C-terminal domain: a case report.

Martoni E, Petrini S, Trabanelli C, Sabatelli P, Urciuolo A, Selvatici R, D'Amico A, Falzarano S, Bertini E, Bonaldo P, Ferlini A, Gualandi F.

BMC Med Genet. 2013 Jun 5;14:59. doi: 10.1186/1471-2350-14-59.

13.

Melanocytes--a novel tool to study mitochondrial dysfunction in Duchenne muscular dystrophy.

Pellegrini C, Zulian A, Gualandi F, Manzati E, Merlini L, Michelini ME, Benassi L, Marmiroli S, Ferlini A, Sabatelli P, Bernardi P, Maraldi NM.

J Cell Physiol. 2013 Jun;228(6):1323-31. doi: 10.1002/jcp.24290.

14.

Cytoskeletal and extracellular matrix alterations in limb girdle muscular dystrophy 2I muscle fibers.

Sabatelli P, Pellegrini C, Faldini C, Merlini L.

Neurol India. 2012 Sep-Oct;60(5):510-1. doi: 10.4103/0028-3886.103200.

15.

Persistent dystrophin protein restoration 90 days after a course of intraperitoneally administered naked 2'OMePS AON and ZM2 NP-AON complexes in mdx mice.

Bassi E, Falzarano S, Fabris M, Gualandi F, Merlini L, Vattemi G, Perrone D, Marchesi E, Sabatelli P, Sparnacci K, Laus M, Bonaldo P, Rimessi P, Braghetta P, Ferlini A.

J Biomed Biotechnol. 2012;2012:897076. doi: 10.1155/2012/897076. Epub 2012 Oct 2.

16.

Antisense-induced messenger depletion corrects a COL6A2 dominant mutation in Ullrich myopathy.

Gualandi F, Manzati E, Sabatelli P, Passarelli C, Bovolenta M, Pellegrini C, Perrone D, Squarzoni S, Pegoraro E, Bonaldo P, Ferlini A.

Hum Gene Ther. 2012 Dec;23(12):1313-8. doi: 10.1089/hum.2012.109. Epub 2012 Nov 6.

17.

Muscle fiber atrophy and regeneration coexist in collagen VI-deficient human muscle: role of calpain-3 and nuclear factor-κB signaling.

Paco S, Ferrer I, Jou C, Cusí V, Corbera J, Torner F, Gualandi F, Sabatelli P, Orozco A, Gómez-Foix AM, Colomer J, Nascimento A, Jimenez-Mallebrera C.

J Neuropathol Exp Neurol. 2012 Oct;71(10):894-906.

18.

Genetic characterization in symptomatic female DMD carriers: lack of relationship between X-inactivation, transcriptional DMD allele balancing and phenotype.

Brioschi S, Gualandi F, Scotton C, Armaroli A, Bovolenta M, Falzarano MS, Sabatelli P, Selvatici R, D'Amico A, Pane M, Ricci G, Siciliano G, Tedeschi S, Pini A, Vercelli L, De Grandis D, Mercuri E, Bertini E, Merlini L, Mongini T, Ferlini A.

BMC Med Genet. 2012 Aug 16;13:73.

19.

Vascular smooth muscle Emilin-1 is a regulator of arteriolar myogenic response and blood pressure.

Litteri G, Carnevale D, D'Urso A, Cifelli G, Braghetta P, Damato A, Bizzotto D, Landolfi A, Ros FD, Sabatelli P, Facchinello N, Maffei A, Volpin D, Colombatti A, Bressan GM, Lembo G.

Arterioscler Thromb Vasc Biol. 2012 Sep;32(9):2178-84. doi: 10.1161/ATVBAHA.112.254664. Epub 2012 Jul 19.

20.

Expression of collagen VI α5 and α6 chains in human muscle and in Duchenne muscular dystrophy-related muscle fibrosis.

Sabatelli P, Gualandi F, Gara SK, Grumati P, Zamparelli A, Martoni E, Pellegrini C, Merlini L, Ferlini A, Bonaldo P, Maraldi NM, Paulsson M, Squarzoni S, Wagener R.

Matrix Biol. 2012 Apr;31(3):187-96. doi: 10.1016/j.matbio.2011.12.003. Epub 2011 Dec 30.

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