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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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Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Castel D ; Sambasivan R ; Tajbakhsh S | 2011Satellite cells play a major role in postnatal growth and regeneration of skeletal muscle. Thus, satellite cells exhibit several attributes of stem cells: quiescence, selfrenewal and regenerative potential. Since all skeletal muscle satellite ce[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Lacourpaille L ; Bouillard K ; Hug F ; Hogrel JY ; Nordez A | 2011Non-invasive measurements are required for monitoring neuromuscular disorders (NDM) and following the effects of rehabilitation/therapy programs. Since it provides localized elastic modulus (i.e. stiffness) measurements, elastographic methods co[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Vincent L ; Féasson L ; Messonnier L | AFM-TELETHON | 2011Sickle cell disease (SCD) is an inherited blood disorder that leads to the production of abnormal haemoglobin (Hb), called HbS. In previous experiments, we have shown that sickle cell trait (SCT) carriers (heterozygous form of the disease, chara[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Vandenburgh H ; Shansky J ; Benesch-Lee F ; Skelly K ; Spinazzola J ; Green S ; Saponjian Y ; Butler Browne G ; Mouly V ; Tseng B | AFM-TELETHON | 2011Skeletal muscle weakness and fatigue disorders affect millions of individuals each year and few treatments are available. A high content physiological drug screening technology was developed using skeletal muscle myoblasts bioengineered into con[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Domi T ; Porrello E ; Velardo D ; Capotondo A ; Triolo D ; Ruegg MA ; Biffi A ; Comi G ; Quattrini A ; Previtali S | 2011CMD is characterized by progressive wasting muscular dystrophy and dysmyelinating neuropathy with variable involvement of the central nervous system, which may lead to severe disability in early childhood. Among this group, laminin 2deficient CM[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Buyse G ; Goemans N ; van den Hauwe M ; Thijs D ; Wei H ; Rummey C ; Meier T ; Mertens L | 2011Background: A 12-month double-blind, randomized, placebo-controlled phase II study in 21 patients with DMD (DELPHI study) has indicated that idebenone (Catena®, 450 mg/d) may improve cardiac and respiratory function in 8-16 year old patients wit[...]Article
Reversal of myotonic dystrophy type 1 MIS-splicing events using truncated-muscle blind-like factor 1
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Tran H ; Lermercier C ; Dhaenens CM ; Fernandez-Gomez F ; Eddarkaoui S ; Begard S ; Obriot H ; Van Brussels E ; Caillierez R ; Buee L ; Charlet Berguerand N ; Schraen-Maschke S ; Déglon N ; Colin M ; Furling D ; Caillet-Boudin ML ; Sergeant N | AFM-TELETHON | 2011Myotonic dystrophy of type I (DM1), the most common form of adult muscular dystrophy, is an autosomal dominant multisystemic inherited disease. The mutation consists of an unstable CTG expansion in the 3'UTR of the DMPK gene, which confers to th[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Stockholm D ; Edom-Vovard F ; Coutant S ; Sanatine P ; Corre C ; Neildez-Nguyen TMA ; Paldi A | AFM-TELETHON | 2011The Neural cell adhesion molecule (NCAM;CD56) is considered as a marker of myogenic cells committed to differentiation. It is commonly used for the enumeration of satellite cells and for the enrichment of myogenic cell population from muscle for[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Lefebvre S ; Renvoisé B ; Verrier ER ; Quérol G | AFM-TELETHON | 2011Infantile muscular atrophy (SMA) is a group of inherited neuromuscular disorders cause by mutations in the Survival Motor Neuron (SMN) gene. SMAs are characterized by the degeneration of the alpha-motorneurons and muscular atrophy. The ubiquitou[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Ben Yaou R ; Beugnet C ; Chikhaoui K ; Hamroun D ; Deburgrave N ; Kaplan JC ; Beroud C ; Bonne G ; Leturcq F | 2011In 1966, A. Emery and F. Dreifuss described a large family from Virginia, where affected males showed an unusual type of X-linked condition characterised by muscular dystrophy with early joint contractures and cardiac disease, thus starting the [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Giagnacovo M ; Malatesta M ; Cardani R ; Pellicciari C ; Meola G | AFM-TELETHON | 2011Myotonic dystrophies (DMs) have highly variable clinical manifestations consisting in muscle weakness and atrophy, and a wide spectrum of extramuscular manifestations. In both DM1 and DM2 forms, expanded nucleotide sequences cause the accumulati[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Vassilopoulos S ; Gentil C ; Laine J ; Buclez PO ; Brodsky F ; Bonne G ; Voit T ; Garcia L ; Pietri Rouxel F ; Bitoun M | AFM-TELETHON | 2011Clathrin CHC17, the ubiquitous clathrin heavy chain encoded on human chromosome 17, is the main component of clathrin coated vesicles (CCV), well characterized for its role in vesicle formation during endocytosis of membrane receptors from the p[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Joanne P ; Ochala J ; Hourde C ; Hadj-Said W ; Mounier R ; Zibroba D ; Sakamoto K ; Agbulut O ; Butler Browne G ; Ferry A | AFM-TELETHON | 2011Dystrophin contributes to force transmission and has a protein-scaffolding role for a variety of proteins (e.g. mechanoreceptor, nNOS, DHPR&) and is thus emerged to play an important role as components of signaling pathways acting in skeletal mu[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Villemeur M ; Biondi O ; Temchenko A ; Marchand A ; Bourg N ; Richard I ; Authier FJ | AFM-TELETHON | 2011Dysferlin is a membrane-anchored protein implicated in plasma membrane repair process. In mammalians, dysferlin deficiency induces persistent sarcolemma disruptions leading to myofiber necrosis and chronic myopathy. Dysferlin myopathy is charact[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Falcone S ; Gache V ; Didier N ; Sassoon D ; Gomes E | 2011Centronuclear myopathies (CNMs) are congenital muscle disorders, described for the first time in 1966 by A. Spiro, characterized morphologically by small fibers with centrally-positioned nuclei. Although, at present, mutations in at least three [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Jessica KM ; Relaix F | AFM-TELETHON | 2011During embryogenesis, the skeletal muscle is constituted by myogenic progenitor cells. But around birth, foetal muscle progenitor cells adopt a satellite cell position, becoming embedded within the basal lamina in close contact to the muscle fib[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Deltour L ; Tajbakhsh S ; Poirier F | 2011Galectins belong to a family of soluble proteins that can be found both extracellularly and intracellularly. In vivo, their localisation is highly regulated depending on physiological situations. Several studies has established that, in addition[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Ratti F ; Ramond F ; Gilquin B ; Zhang Y ; Khochbin S ; Matthias P ; Méjat A ; Schaeffer L | AFM-TELETHON | 2011Skeletal muscle atrophy is a widespread and debilitating disease that mainly results from the massive degradation of muscle proteins, together with an inhibition of regenerating activity. The main occurring event is the down-regulation of the ak[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Picard B ; Anne B ; Cassar-Malek I ; Chelh I ; Cottin P ; Gabillard JC ; Hadj Sassi A ; Leibovitch S ; Rodriguez J ; Seilliez I | AFM-TELETHON | 2011The control of muscle mass is determined by a dynamic balance of anabolic and catabolic processes. In the last decade, myostatin, a member of the TGFuperfamily, has emerged as a key factor in muscle growth regulation. The importance of myostatin[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Weiss J ; Bismuth J ; Eymard B ; Berrih-Aknin S ; Le Panse R | AFM-TELETHON | 2011Myasthenia Gravis (MG) with anti-acetylcholine receptor antibodies is commonly associated with thymic hyperplasia characterized by the presence of germinal centers containing B cells producing pathogenic antibodies.Our analysis of hyperplastic t[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Christodoulou-Vafeiadou E ; Favier M ; Navière A ; Maire P ; Guillet-Deniau I | 2011Intramyocellular lipid deposition contributes to muscle insulin resistance, particularly in obese, type 2 diabetic patients, or in patients with myotonic dystrophy. On the other hand, disruption of Wnt signaling caused transdifferentiation of my[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Boutonnet C ; Bello NF ; Lamsoul I ; Métais A ; Mouly V ; Lutz P ; Moog-Lutz C | 2011Our work is based on human primary myoblast cultures derived from single satellite cells. In human, as in other species, it is well established that myoblast differentiation is mainly controlled by two families of transcription factors, the Myog[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Grassi F ; Bigi R ; Deflorio C ; Fucile S | AFM-TELETHON | 2011In slow-channel congenital myasthenia (CM), excessive Ca2+ influx through mutant endplate acetylcholine receptor (AChR) channels leads to endplate degeneration and myasthenic symptoms. The high Ca2+ permeability of human AChR-channels, which is [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Tajsharghi H | AFM-TELETHON | 2011Saba Abdul-Hussein, Homa Tajsharghi Department of Pathology, Institute of Biomedicine, University of Gothenburg, 413 45 Gothenburg, Sweden In vertebrates skeletal muscle is found throughout the body and forms during the entire life span. Adult s[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Castets P ; Bertrand A ; Beuvin M ; Ferry A ; Grand FL ; Castets M ; Chazot G ; Rederstorff M ; Krol A ; Lescure A ; Romero NB ; Guicheney P ; Allamand V | 2011In humans, selenoprotein N (SelN) deficiency, due to mutations in the SEPN1 gene, causes a group of inherited neuromuscular disorders termed SEPN1-Related Myopathies. These congenital diseases are characterized by an early onset generalized musc[...]