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Long-term microdystrophin gene therapy is effective in a canine model of Duchenne muscular dystrophy.

Le Guiner C, Servais L, Montus M, et al.
Nature communications, 2017, 8, 15 p.

Notice complète Revue : Nature communications, 8 Titre : Long-term microdystrophin gene therapy is effective in a canine model of Duchenne muscular dystrophy. Type de document : Article Auteurs : Le Guiner C ; Servais L ; Montus M ; Larcher F ; Fraysse B ; Moullec S ; Allais M ; François V ; Dutilleul M ; Malerba A ; Koo T ; Thibaut JL ; Matot B ; Devaux M ; Le Duff J ; Deschamps JY ; Barthelemy I ; Blot S ; Testault I ; Wahbi K ; Ederhy S ; Martin S ; Veron P ; Georger C ; Athanasopoulos T ; Masurier C ; Mingozzi F ; Carlier P ; Gjata B ; Hogrel JY ; Adjali O ; Mavilio F ; Voit T ; Moullier P ; Dickson G Année de publication : 25/07/2017 Pages : 15 p. Langues : Anglais (eng) Mots-clés : chien grmd ; chien modèle ; dystrophie musculaire de Duchenne ; dystrophine ; essai préclinique ; force musculaire ; gène minidystrophine ; histopathologie musculaire ; injection intramusculaire ; injection intraveineuse ; IRM ; muscle squelettique ; thérapie génique ; vecteur adénoassocié Pubmed / DOI : DOI : 10.1038/ncomms16105 / Pubmed : 28742067 En ligne : http://www.ncbi.nlm.nih.gov/pubmed/28742067

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Gait characterization in golden retriever muscular dystrophy dogs using linear discriminant analysis.

Fraysse B, Barthelemy I, Qannari EM, et al.
BMC musculoskeletal disorders, 2017, 18, 153, 9 p.

Notice complète Revue : BMC musculoskeletal disorders, 18, 153 Titre : Gait characterization in golden retriever muscular dystrophy dogs using linear discriminant analysis. Type de document : Article Auteurs : Fraysse B ; Barthelemy I ; Qannari EM ; Rouger K ; Thorin C ; Blot S ; Le Guiner C ; Cherel Y ; Hogrel JY Année de publication : 12/04/2017 Pages : 9 p. Langues : Anglais (eng) Mots-clés : accélérométrie ; analyse de la marche ; chien grmd ; essai préclinique ; modélisation Pubmed / DOI : DOI : 10.1186/s12891-017-1494-4 / Pubmed : 28403854 En ligne : http://www.ncbi.nlm.nih.gov/pubmed/28403854

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Calcium homeostasis alterations in a mouse model of the Dynamin 2-related centronuclear myopathy.

Fraysse B, Guicheney P, Bitoun M
Biology open, 2016, 5, 11, p. 1691-1696

Notice complète Revue : Biology open, 5, 11 Titre : Calcium homeostasis alterations in a mouse model of the Dynamin 2-related centronuclear myopathy. Type de document : Article Auteurs : Fraysse B ; Guicheney P ; Bitoun M Année de publication : 15/11/2016 Pages : p. 1691-1696 Langues : Anglais (eng) Pubmed / DOI : DOI : 10.1242/bio.020263 / Pubmed : 27870637 En ligne : http://www.ncbi.nlm.nih.gov/pubmed/27870637

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Dystrophin restoration therapy improves both the reduced excitability and the force drop induced by lengthening contractions in dystrophic mdx skeletal muscle.

Roy P, Rau F, Ochala J, et al.
Skeletal Muscle, 2016, 6, sp

Notice complète Revue : Skeletal Muscle, 6 Titre : Dystrophin restoration therapy improves both the reduced excitability and the force drop induced by lengthening contractions in dystrophic mdx skeletal muscle. Type de document : Article Auteurs : Roy P ; Rau F ; Ochala J ; Messéant J ; Fraysse B ; Lainé J ; Agbulut O ; Butler-Browne G ; Furling D ; Ferry A Editeur : England Année de publication : 20/07/2016 Pages : sp Langues : Anglais (eng) Résumé : BACKGROUND: The greater susceptibility to contraction-induced skeletal muscle injury (fragility) is an important dystrophic feature and tool for testing preclinic dystrophin-based therapies for Duchenne muscular dystrophy. However, how these therapies reduce the muscle fragility is not clear. METHODS: To address this question, we first determined the event(s) of the excitation-contraction cycle which is/are altered following lengthening (eccentric) contractions in the mdx muscle. RESULTS: We found that the immediate force drop following lengthening contractions, a widely used measure of muscle fragility, was associated with reduced muscle excitability. Moreover, the force drop can be mimicked by an experimental reduction in muscle excitation of uninjured muscle. Furthermore, the force drop was not related to major neuromuscular transmission failure, excitation-contraction uncoupling, and myofibrillar impairment. Secondly, and importantly, the re-expression of functional truncated dystrophin in the muscle of mdx mice using an exon skipping strategy partially prevented the reductions in both force drop and muscle excitability following lengthening contractions. CONCLUSION: We demonstrated for the first time that (i) the increased susceptibility to contraction-induced muscle injury in mdx mice is mainly attributable to reduced muscle excitability; (ii) dystrophin-based therapy improves fragility of the dystrophic skeletal muscle by preventing reduction in muscle excitability. Pubmed / DOI : DOI : 10.1186/s13395-016-0096-4 / Pubmed : 27441081 En ligne : http://www.ncbi.nlm.nih.gov/pubmed/27441081

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Mechanical Overloading Increases Maximal Force and Reduces Fragility in Hind Limb Skeletal Muscle from Mdx Mouse.

Ferry A, Parlakian A, Joanne P, et al.
The American journal of pathology, 2015, 185, 7, p. 2012-2024

Notice complète Revue : The American journal of pathology, 185, 7 Titre : Mechanical Overloading Increases Maximal Force and Reduces Fragility in Hind Limb Skeletal Muscle from Mdx Mouse. Type de document : Article Auteurs : Ferry A ; Parlakian A ; Joanne P ; Fraysse B ; Mgrditchian T ; Roy P ; Furling D ; Butler-Browne G ; Agbulut O Année de publication : 07/2015 Pages : p. 2012-2024 ISBN/ISSN/EAN : 26009153 Langues : Anglais (eng) Résumé : There is fear that mechanical overloading (OVL; ie, high-force contractions) accelerates Duchenne muscular dystrophy. Herein, we determined whether short-term OVL combined with wheel running, short-term OVL combined with irradiation, and long-term OVL are detrimental for hind limb mdx mouse muscle, a murine model of Duchene muscular dystrophy exhibiting milder dystrophic features. OVL was induced by the surgical ablation of the synergic muscles of the plantaris muscle, a fast muscle susceptible to contraction-induced muscle damage in mdx mice. We found that short-term OVL combined with wheel and long-term OVL did not worsen the deficit in specific maximal force (ie, absolute maximal force normalized to muscle size) and histological markers of muscle damage (percentage of regenerating fibers and fibrosis) in mdx mice. Moreover, long-term OVL did not increase the alteration in calcium homeostasis and did not deplete muscle cell progenitors expressing Pax 7 in mdx mice. Irradiation before short-term OVL, which is believed to inhibit muscle regeneration, was not more detrimental to mdx than control mice. Interestingly, short-term OVL combined with wheel and long-term OVL markedly improved the susceptibility to contraction-induced damage, increased absolute maximal force, induced hypertrophy, and promoted a slower, more oxidative phenotype. Together, these findings indicate that OVL is beneficial to mdx muscle, and muscle regeneration does not mask the potentially detrimental effect of OVL. Pubmed / DOI : DOI : 10.1016/j.ajpath.2015.03.027 / Pubmed : 26009153 En ligne : http://www.ncbi.nlm.nih.gov/pubmed/26009153

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Acute effect of androgens on maximal force-generating capacity and electrically evoked calcium transient in mouse skeletal muscles.

Fraysse B, Vignaud A, Fane B, et al.
Steroids, 2014, 87, p. 6-11

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Assessment of a symptomatic Duchenne muscular dystrophy carrier 20 years after myoblast transplantation from her asymptomatic identical twin sister

Hogrel JY, Zagnoli F, Canal A, et al.
Neuromuscular disorders : NMD, 2013, 23, 7, p 575

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Increased myofilament Ca2+ sensitivity and diastolic dysfunction as early consequences of Mybpc3 mutation in heterozygous knock-in mice

Fraysse B, Weinberger F, Bardswell S, et al.
Journal of molecular and cellular cardiology, 2012, 52, p. 1299-1307

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A centronuclear myopathy-dynamin 2 mutation impairs autophagy in mice

Durieux AC, Vassilopoulos S, Lainé J, et al.
Traffic, 2012, 13, 6, p. 869-879

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A centronuclear myopathy-dynamin2 mutation impairs autophagy in mice

Durieux AC, Vassilopoulos S, Lainé J, et al.
Congrès : 4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France))
2011, p. 59

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Study of cytosolic calcium domains in delta-sarcoglycan deficient cardiomyocytes (poster)

Fraysse B, Ragot H, Delmasure A, et al.
Congrès : 4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France))
2011, p. 51

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Ca2+ overload and mitochondrial permeability transition pore activation in living {delta}-sarcoglycan deficient cardiomyocytes

Fraysse B, Nagi SM, Boher B, et al.
American journal of physiology. Cell physiology, 2010, 299, 3, p. C706-713

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A centronuclear myopathy-dynamin 2 mutation impairs skeletal muscle structure and function in mice

Durieux AC, Vignaud A, Prudhon B, et al.
Human molecular genetics, 2010, 19, 24, p. 4820-4836

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Oxidative stress in SEPN1-related myopathy : from pathophysiology to treatment

Arbogast S, Beuvin M, Fraysse B, et al.
Annals of neurology, 2009, 65, 6, p. 677-686

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Multiple pathological events in exercised dystrophic mdx mice are targeted by pentoxifylline: outcome of a large array of in vivo and ex vivo tests

Burdi R, Rolland JF, Fraysse B, et al.
Journal of applied physiology, 2009, 106, 4, p. 1311-1324

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Nonsense-Mediated mRNA Decay and Ubiquitin-Proteasome System Regulate Cardiac Myosin-Binding Protein C Mutant Levels in Cardiomyopathic Mice

Vignier N, Schlossarek S, Fraysse B, et al.
Circulation research, 2009, 105, 3, p. 239-248

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Alterations of the CA2+ homeostasis correlate dystrophic phenotype severity in native delta-sarcoglycan deficient skeletal muscles

Fraysse B, Salmon A, Nagi S, et al.
Congrès : Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France)
2008, p. 194

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Role of tumour necrosis factor alpha, but not of cyclo-oxygenase-2-derived eicosanoids, on functional and morphological indices of dystrophic progression in mdx mice: a pharmacological approach

Nico B, Burdi R, Liantonio A, et al.
Neuropathology and applied neurobiology, 2007, 33, 3, p. 344-359

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A multidisciplinary evaluation of the effectiveness of cyclosporine A in dystrophic Mdx mice

de Luca A, Nico B, Liantonio A, et al.
American journal of pathology (The), 2005, 166, 2, p. 477-489

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Growth hormone secretagogues tune intracellular calcium in native rat skeletal muscle fibers (abstract : congrès international de Myologie, 2005)

Fraysse B, Liantonio A, Giannuzzi V, et al.
Congrès : Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France)
2005, p. 297

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A new mouse model of familial hypertrophic cardiomyopathy exhibits instability of the E258K mutant cardiac myosin-binding protein C (abstract : congrès international de Myologie, 2005)

Vignier N, Fraysse B, Pointu H, et al.
Congrès : Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France)
2005, p. 204

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