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Congrès: Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) (26-30 mai 2008)
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Documents disponibles provenant de ce congrès (565)
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Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Notarnicola C ; Le Guen L ; de Santa Barbara P | 2008Despite significant advances in the description of molecular controls of gut development in different animal models, little works have been done on the pathways involved during visceral smooth muscle cell differentiation. This differentiation is[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Saillour Y ; Mercier S ; Avril-Delplanque A ; Daoud F ; Nusbaum P ; Leturcq F ; Kaplan JC ; Dreyfus PA ; Cossee M ; Garcia L ; Bienvenu T ; Chelly J | 2008Duchenne Muscular Dystrophy (DMD) is the most severe form of dystrophinopathy, in which null mutations in the DMD gene (mostly frameshifting deletions, and nonsense point mutations) result in the complete absence of dystrophin. A milder phenotyp[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Salort-Campana E ; Figarella-Branger D ; Pellissier JF ; Krahn M ; Pouget J | 2008OBJECTIVE: To determine the distribution of subtypes of Limb-Girdle Muscular Dystrophy (LGMD) among patients in South-East of France. To determine the proportion of patients in whom a molecular diagnosis was available. BACKGROUND: LGMD are a het[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Benhassine T ; Hamadouche T ; Assami S ; Makri S ; Chaouch M ; Tazir M | 2008Proximal spinal muscular atrophy (SMA) are a group of motor neuropathies characterized by the degeneration of spinal montoneurons leading to muscular paralysis with muscular atrophy. They are the second most fatal autosomal recessive disease, wi[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Bonello-Palot N ; Latour P ; Martini N ; Mayençon M ; Pêcheux C ; Mégarbané A ; Attarian S ; Pouget J ; Bernard R | 2008Charcot-Marie-Tooth neuropathies (CMT), also known as hereditary motor and sensory neuropathies (HMSN), are a group of genetically and clinically heterogeneous diseases of the peripheral nervous system. 40 genes and more than 60 loci have been i[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Wan L ; Kasim M ; Wang C ; Cho S ; Bachorik J ; Cho Y ; Dreyfuss G | 2008The SMN complex, comprised of the survival of motor neurons (SMN) protein and the Gemins, plays a central role in cellular RNA metabolism. The SMN complex is essential in all cells and functions as a molecular assembly machine for the biogenesis[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Sifi Y ; Sifi K ; Bestandji K ; Boulefkhad A ; Abadi N ; Benlatreche C ; Hamri A | 2008Introduction: Spinal muscular atrophy (SMA) is one of the most common autosomal recessive disorders, characterized by degeneration of anterior horn cells in the spinal cord, and leads to progressive muscular weakness and atrophy In the present s[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Hinard V ; Leroy M ; Bader CR ; Bernheim L | 2008Myoblasts are mononucleated cells that fuse together to form skeletal muscle fibers. We have shown previously that human myoblast differentiation requires a hyperpolarization of the resting membrane potential to take place. This hyperpolarizatio[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Scicchitano B ; Toschi A ; Murfuni I ; Molinaro M ; Adamo S | 2008The maintenance of a working skeletal musculature is conferred by its remarkable ability to regenerate after mechanical or pathological injury. However muscle atrophies are characterized by the progressive loss of muscle tissue due to alteration[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Hamadouche T ; Delague V ; de Sandre-Giovannoli A ; Bernard R ; Génin E ; Benhassine T ; Tazir M ; Chaouch M ; Nouioua S ; Kassouri N ; Amer E ; Kheddoud W ; Vallat JM ; Grid D | 2008Hereditary motor and sensory neuropathies (HMSN), commonly referred to as Charcot-Marie-Tooth disease (CMT), are among the most common inherited neurological diseases, with an overall prevalence of about 1-4/10,000. While all modes of inheritanc[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Vauchez K ; Baligand C ; Vilquin JT ; Fiszman M ; Carlier P | 2008Purpose: High spatial resolution and non-invasiveness feature NMR imaging, and may allow longitudinal assessment of cell therapies. However, cells must be pre-loaded with an appropriate NMR contrast agent (CA). We compared the ability of two cla[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Mignard C ; Charlin C ; Mignard D ; Roelens P | 2008In Reunion Island, we followed fifty ataxic patients every six months during eight years, from November 1999 to July 2007, using the same protocol. 1 The work protocol: Patients were included regardless of the stage of their disease. They were r[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Fougerousse F ; Giannesini B ; Durand M ; Guerchet N ; Bendahan D ; Cozzone PJ ; Richard I | 2008We aim at characterizing muscle function in a cohort of different animal models for LGMD2 using mechanical parameters in several isolated muscles. Non invasive techniques will also be applied such as grip, escape or wire tests and measurements o[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Amendola J ; Durand J | 2008The early mechanisms leading to the progressive loss of motoneurons during ALS are still unknown. Using the in vitro preparation of brainstem/spinal cord isolated from wild-type and SOD1G85R transgenic mice (ALS model), we have recently reported[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Galan L ; Vela A ; Guerrero A ; Taxonera C ; Matias-Guiu J | 2008INTRODUCTION Inflammatory Bowel Disease (IBD) presents many neurological complications. Up to 3% of patients with IBD have neurological involvement (Thromboembolic phenomena, myelopathy, myopathy, multiple sclerosis and various neuropathies). Ju[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Singh S ; Aurangabadkar K ; Goyal V ; Sharma MC ; Sarkar C ; Behari M | 2008Background –Muscle histopathology may be inconclusive because of the patchy muscle involvement in inflammatory myopathies. Imaging techniques may be of use in selecting the exact site for biopsy. Objective – To evaluate the role of muscle Magnet[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Siala O ; Nacim L ; Chahnez T ; Madeleine M ; Ahmed R ; Faouzi B ; Faiza F | 2008Studying the impact of genomic variations on mRNA metabolism became a central issue to better understand the biological significance of diseases. The objective of our study was to understand the effect of genomic mutations in LAMA2 gene on mRNA [...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Friedrich A ; Garnier N ; Blandin G ; Bettler E ; Richard I ; Moulinier L ; Poch O | 2008The MS2PH project (from Structural Mutation to Human Pathologies Phenotypes) aims at investigating how mutations impact protein structures and to which extent this change could affect pathological phenotypes. This project is part of the Decrypth[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Brule C ; Dargelos E ; Cottin P ; Poussard S | 2008The calcium-dependent proteolytic system is composed of cysteine proteases named calpains. They are ubiquitous or tissue-specific enzymes and the two best characterised isoforms are the ubiquitously expressed µ- and m-calpains. Besides its regul[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Mouisel E ; Hourde C ; Vignaud A ; Butler Browne G ; Ferry A | 2008Aging results in a deterioration of muscle structure and function in dystrophic mdx mice lacking dystrophin. In the present study we wanted to know whether this aggravation is associated with an age related decline in regenerative capacity. Both[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Arsic N ; Mamaeva D ; Lamb N ; Fernandez A | 2008Stem cells with the ability to differentiate in specialized cell types can be extracted from a wide array of adult tissues including skeletal muscle. Here we have characterized a population of stem cells from skeletal muscle that can be reproduc[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Vulin A ; Barthelemy I ; Goyenvalle A ; Lorain S ; Thibaud JL ; Bertoldi D ; Carlier P ; Dreyfus P ; Danos O ; Blot S ; Garcia L | 2008Duchenne Muscular Dystrophy (DMD) is an X-linked recessive disorder due to mutations in the gene encoding dystrophin. Most of mutations consist in large deletions, although their size is not correlated with the severity of the phenotype. Out-of-[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Vulin A ; Barthelemy I ; Dreyfus P ; Blot S ; Garcia L | 2008Duchenne Muscular Dystrophy (DMD) is an X-linked recessive disorder due to mutations in the gene that encodes dystrophin. Most of these mutations consist in large genomic deletions, although their extent is not directly correlated with the sever[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Mouisel E ; Vulin A ; Hourde C ; Dumonceaux J ; Relaix F ; Garcia L ; Amthor H | 2008Previously, we have shown that null mutation of myostatin resulted in muscle fibre hypertrophy that is independent of satellite cell activity. Here, we investigated the effect of myostatin blockade in mature mouse muscle. Myostatin was blocked a[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Laurent E ; Borel P ; Hogrel JY ; Bousquet N ; Fougerousse F ; Stockholm D ; Calpain Study Group | 2008In the Natural History of Calpainopathies study (F Fougerousse's communication), 37 patients were subjected to Computed Tomography (CT) scans of lower limbs at the beginning of the investigation and 2 years later. The objective was to quantify m[...]