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Congrès: 4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) (9-13 mai 2011)
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Documents disponibles provenant de ce congrès (480)
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4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Piekuse L ; Lce B ; Bauze D ; Pronina N ; Daneberga Z ; Krumina A | AFM-TELETHON | 2011Case report - girl 4y of age had genetic consultation due to slight mental retardation, speech delay, autistic behavior, stereotypic movements and not developed fine motor skills. In blood biochemical investigation there were discovered slightly[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Hubert JF ; Moore S ; Delalande O ; Chéron A ; Bennett L ; Kupski W ; El-Baba M ; Le Rumeur E ; Ascadi G | 2011Missense mutations in the dystrophin gene often lead to Becker muscular dystrophy (BMD) but genotype/phenotype correlation is difficult to establish. Amino acid substitutions even in the repeat region of dystrophin may disrupt binding capacities[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Rafai MA ; Ibnkhribchia R ; Boulaajaj F ; Oumari S ; Manaf S ; El Moutawakil B ; Slassi I | AFM-TELETHON | 2011Introduction: Idiopathic orbital myositis is a rare entity, considered a sub-group of the orbit's Inflammatory pseudotumori. Cases report: Case 1: A 44 years old woman admitted for bilateral orbital pain, associated periorbital oedema and diplop[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Loureiro De Sousa P ; Vignaud A ; Caldas de Almeida Araujo E ; Carlier PG | AFM-TELETHON | 2011Background. There is a strong need of non-invasive outcome measures for monitoring the natural progression of muscle disorders, particularly at the early stages of involvement. In this context, quantitative NMR imaging might provide objective ma[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Hanif M ; Pelin K ; Gronholm M ; Wallgren-Pettersson C | 2011The core objective of this study is to help elucidate the functional pathogenesis of nemaline myopathy and related disorders caused by mutations in the nebulin gene (NEB). Nebulin is a giant actin-binding protein which is located in the I-band o[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Fréret M ; Drouot L ; Ahmed Lecheheb S ; Dauly C ; Cosette P ; Authier FJ ; Boyer O | AFM-TELETHON | 2011Normal muscle fibers do not express detectable levels of class I major histocompatibility complex (MHC-I). In contrast, high-level expression of MHC-I is a hallmark of muscle autoimmune diseases such as polymyositis, inclusion-body myositis or d[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Cacheux M ; Fauré J ; Brocard J ; Monnier N ; Lunardi J ; Marty I | 2011Muscle contraction is achieved when an efficient excitation signal at the plasma membrane triggers intracellular calcium release. This process called "excitationcontraction (E-C) coupling" relies on a multimolecular protein complex, the calcium [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Portilho D ; Mamchaoui K ; Lanzarini C ; Capri M ; Salvioli S ; Franceschi C ; van der Maarel S ; Butler Browne G ; Dumonceaux J | AFM-TELETHON | 2011Facioscapulohumeral dystrophy (FSHD) is the third most common muscular dystrophy and it is characterized by progressive weakness and atrophy of the facial and shoulder girdle muscles. FSHD is caused by deletion of most copies of the 3.3-kb subte[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Galy A ; Sambasivan R ; Yao R ; Kissenpfennig A ; Van Wittenberghe L ; Paldi A ; Gayraud-Morel B ; Guenou H ; Malissen B ; Tajbakhsh S | AFM-TELETHON | 2011INTRODUCTION: Muscle-derived cells are able to differentiate towards osteogenic, chondrogenic or adipogenic lineage, in addition of their myogenic potential. This raises many biological and clinical questions. The cellular bases and the role of [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Lepper C ; Partridge T ; Chen-Ming F | AFM-TELETHON | 2011Skeletal muscle tissue is prone to damage from acute physical trauma such as sport injuries as well as from daily wear and tear: this is managed by its tremendous capacity to repair itself. Cell transplantation and lineage tracing studies have d[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Kostallari E ; Lafuste P ; Baba-Amer Y ; Gherardi RK | 2011Sublaminar location has been the cornerstone of both definition and identification of muscle satellite cells (mSCs), but little attention has been paid to the anatomic organization of the mSC niche, and interplays of mSCs with their neighborhood[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Sarrazin N ; Bangratz M ; Devaux J ; Zambroni D ; Echaniz-Laguna JA ; Rene F ; Boerio D ; Davoine CS ; Fontaine B ; Feltri ML ; Benoit E ; Nicole S | 2011Schwartz-Jampel syndrome (SJS) is a recessive disorder characterized by spontaneous activity in the rest EMG that may result from peripheral nerve hyperexcitability (PNH). SJS results from a lack of perlecan, the major proteoglycan of basement m[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Daussin F ; Godin R ; Ascah A ; Deschênes S ; Petrof B ; Burelle Y | 2011We examined the mitochondrial phenotype in skeletal muscle in the early phase of Duchenne muscular dystrophy (DMD), and determined whether upregulation of mitochondrial biogenesis via PGC-1_ transfection is beneficial in the mdx mice, a murine m[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Jedrzejowska M ; Milewski M ; Zimowski J ; Kostera-Pruszczyk A ; Jurek M ; Hausmanowa-Petrusewicz I | AFM-TELETHON | 2011In this report we summarize the results of the work on the phenotype, epidemiology and molecular genetics of Polish cases of spinal muscular atrophy caused by SMN1 mutations.Biallelic loss of exon 7 of the SMN1 gene, the most frequent SMA mutati[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Briggs D ; Morgan J ; Boldrin L | 2011Satellite cells are the principal muscle stem cell, however not all satellite cells contribute equally to muscle regeneration. It is thought that there is a sub-population of satellite cells which is more 'stem cell like' than others. We aim to [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Viollet L ; Lewelt A ; Alvarez JC ; Reyna S ; Stoddard G ; Etheridge S ; LaSalle B ; Swoboda K | AFM-TELETHON | 2011The aim of this project is to determine whether 3,4 diaminopyridine (3,4DAP) is a viable treatment candidate for spinal muscular atrophy (SMA). Aminopyridines are voltage dependent potassium channel inhibitors that increase the duration and the [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Helmbacher F ; Caruso N ; Herberth B ; Bartoli M ; Dumonceaux J ; Lebossé M ; Maina F | AFM-TELETHON | 2011Generation of skeletal muscles with forms adapted to their function is essential for normal movement, and involves developmental mechanisms such as those regulating muscle diversification and migration. Understanding these developmental processe[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Roig-Borrellas A ; Diaz-Ramos MA ; García-Melero A ; Lopez-Alemany R | AFM-TELETHON | 2011Plasminogen activation system (PA) plays an important role in the degradation of extracellular matrix components. Plasmin (Pli), the activated form of plasminogen (Plg), is implicated in several biological processes such as tissue remodelating a[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Cassinelli LM ; Maciotta S ; Gandolfi F ; Forcato M ; Bicciato S ; Meregalli M ; Torrente Y | 2011Duchenne muscular dystrophy (DMD) is a common X-linked disease characterized by frameshift mutations in the dystrophin gene. Among the molecular mechanisms potentially involved in DMD, we focused our attention on microRNAs (miRNAs) a new class o[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Pinto Mariz F ; Barthelemy I ; Yada E ; Voit T ; Silva-Barbosa SD ; Savino W ; Butler Browne G ; Blot S | 2011It is well known that the disease course in Duchenne muscular dystrophy (DMD) patients is heterogeneous, varying from patient to patient. Such heterogeneity is also seen in the Golden Retriever Muscular Dystrophy (GRMD) dogs. This is a drawback [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Wahbi K ; Meune C ; Bécane HM ; Laforet P ; Behin A ; Stojkovic T ; Radvanyi-Hoffman H ; Eymard B ; Duboc D | AFM-TELETHON | 2011Background: Permanent pacing is recommended in patients with myotonic dystrophy type 1 (DM1) for the prevention of sudden cardiac death. However its impact on mortality is unknown.Methods: We retrospectively analyzed medical information of DM1 p[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Diguet N ; Mallat Y ; Ladouce R ; Clodic G ; Tritsch E ; Blanc J ; Larcher JC ; Delcayre C ; Samuel JL ; Friguet B ; Bolbach G ; Li Z ; Mericskay M | 2011RATIONALE: Alterations in the balance between sarcomeric and extra-sarcomeric cytoskeleton as well as energetic proteins are involved in the remodelling of cardiomyocytes cytoarchitecture in dilated cardiomyopathy (DCM). OBJECTIVE: Inactivation [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Benedetti S ; Magagnotti C ; Bachi A ; Zerbini G ; Fattore E ; Riba M ; Previtali S ; M.Ferrari ; Andolfo A | 2011Lamins A/C, encoded by the LMNA gene, are intermediate filaments of the nuclear lamina playing multiple roles in nuclear integrity, chromatin organization and transcriptional control. Defects in the LMNA gene have been associated with a growing [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Denard J ; Beley C ; Kotin R ; Samulski J ; Moullier P ; Voit T ; Garcia L ; Svinartchouk F | 2011Despite the well-established safety and efficacy of rAAV vectors for in vivo gene transfer, there is still little information concerning the fate of vectors after systemic delivery. By using a proteomic approach, we screened for serum proteins i[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Barthelemy I ; Thibaud JL ; Aguilar P ; Le Chevoir M ; Blot S | 2011Most of DMD (Duchenne muscular dystrophy) patients die from respiratory failure. Their canine homologues, GRMD (Golden retriever muscular dystrophy) dogs, also develop respiratory dysfunction. The evaluation of this function seems essential duri[...]