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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 (374)
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4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Decostre V ; Vignaud A ; Gourdon G ; Hogrel JY | 2011Introduction. The aim of this study was to set up an in vivo and non-invasive follow-up of the skeletal muscle function in small rodents. In this purpose we developed ahome-made torquemeter based on previous studies in the literature. The reprod[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Villa C ; Farini A ; Erratico S ; Belicchi M ; Meregalli M ; Fiori F ; Rustichelli F ; Torrente Y | 2011Cell therapy is an emerging approach of regenerative medicine with significant efforts in clinical areas. Stem cells cannot be easily observed directly when injected systemically, and, therefore, their behaviors need to be visualized indirectly.[...]Article
Increased cation entry throught rpc1 is mediated by PLC/PKC pathway in dystrophin-deficient myotubes
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Harisseh R ; Sabourin J ; Magaud C ; Déliot N ; Cognard C ; Constantin B | 2011Dystrophin is an integral structural component of skeletal and cardiac muscles. It provides mechanical stability during muscle contraction and has a crucial signaling role. The absence of dystrophin in DMD leads to calcium homeostasis dysregulat[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Cowling B ; Amoasii L ; Toussaint A ; Koebel P ; Ferry A ; Davignon L ; Nishino I ; Mandel JL ; Laporte J | 2011Dynamin 2 (DNM2) is a large GTPase implicated in many cellular functions including cytoskeleton regulation and endocytosis. Whilst ubiquitously expressed, DNM2 was found mutated in two genetic disorders affecting different tissues: Autosomal Dom[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Penttila S ; Palmio J ; Suominen T ; Udd B | Institut de Myologie AIM | 2011Recently, recessive mutations in ANO5 gene have been shown to be a major cause of limb-girdle and other types of muscular dystrophy. According to recent studies, LGMD2L is one of the most common LGMDs in Europe, its prevalence being similar to t[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Noviello M ; SaverioTedesco F ; Bondanza A ; Tonlorenzi R ; Gerli M ; Peretti G ; Bonini C | 2011Duchenne muscular dystrophy (DMD) is the most severe form of genetic muscular dystrophies. Albeit antinflammatory therapy has been shown to ameliorate disease course, at present DMD remains an incurable disease. Over the last few years, differen[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Salort-Campana E ; Bernard R ; Nguyen K ; Sole G ; Niederhauser J ; Jouve E ; Fourquet I ; Fabre E ; Ollagnon E ; Sacconi S ; Echaniz-Laguna JA ; Duvocelle A ; Vial C ; Arne-Bes MC ; Desnuelle C ; Tranchant C ; Kuntzer T ; Ferrer X ; Pénisson-Besnier I ; Pouget J ; Attarian S | 2011BACKGROUNDFacioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant myopathy associated with contraction of the subtelomeric D4Z4 repeat array on chromosome 4q.There is a marked inter and intra-familial heterogeneity in its clinical[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Gutierrez Cortés N ; Börlin M ; Taanman JW ; Letellier T ; Rocher C | 2011Mitochondrial disorders belong, as a group, to the most common types of genetic disorders. Many of the patients carry either a mutation in a large fraction of theirmitochondrial DNA (mtDNA) molecules or show a depletion of mtDNA. In both cases, [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Repele A ; Piccoli M ; Franzin C ; Urbani L ; Zanon GF ; Milan G ; Vettor R ; Basso G ; Pozzobon M | AFM-TELETHON | 2011Oxygen has been demonstrated to influence proliferation and myotubes differentiation. However, the mechanism of influence is mostly unknown. We have recently reported that satellite cells may be comprised of two distinct populations of cells dis[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Lopez-Alemany R ; Roig-Borrellas A ; Diaz-Ramos MA ; Llorens A ; García-Melero A ; Puigivila M | 2011The epidermal growth factor receptor (EGFR) is a transmembrane protein, of the ErbB family of tyrosine kinase receptors. It is considered a pleitropic signaler, usually associated to survival signal pathways. Overexpression of EGFR has been rela[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Muchir A ; Wu W ; Bonne G ; Worman H | 2011Autosomal dominant Emery-Dreifuss muscular dystrophy (EDMD) is caused by mutations in the LMNA gene encoding A-type lamins, protein building blocks of the nuclear lamina. EDMD is characterized by muscle weakness and wasting in a humeroperoneal d[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Ameziane-Le Hir S ; Chéron A ; Hubert JF ; Le Rumeur E ; Raguénès-Nicol C | 2011Duchenne and Becker muscular dystrophy (DMD and BMD respectively) are caused by mutations of the dystrophin's gene coding for a skeletal muscles protein included in the dystrophin-glycoprotein sarcolemmal complex. Whereas complete deficit in dys[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Benard R ; Giocanty-Auregan A ; Chaine G ; El-Mathari B ; Sahel JA ; Rendon A ; R.Tadayoni | 2011Purpose: To explore the interactions between Dystrophin protein 71 (Dp71), a sub-membranous cytoskeleton protein, and the blood retinal barrier (BRB), and a possible protective effect of dexamethasone through the regulation of Dp71. Methods: An [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Sarkis J ; Legrand B ; Chéron A ; Robert E ; Dupont D ; Jardin J ; Le Rumeur E ; Hubert JF ; Vié V | 2011Dystrophin is essential for skeletal muscle function and confers resistance to the sarcolemma by interacting with cytoskeletal and membrane partners. We previously showed that a large part of the rod domain of dystrophin interacts with membrane [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Gulati S ; Kochar G ; Kabra SK ; Kabra M ; Yadav S ; Pandey RM | 2011Background : Corticosteroids are effective in delaying the loss of muscle strength leading to prolongation of ambulation in patients with Duchenne muscular dystrophy (DMD). The recommended dose for prednisone in ambulatory boys is daily 075 mg/k[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Cherel Y ; Larcher T ; Deschamps JY ; Dutilleul M ; Guigand L ; Le Guiner C ; François V ; Montus M ; Servais L ; Voit T ; Moullier P | 2011In Duchenne Muscular Dystrophy (DMD) the selective removal by exon skipping of exons flanking an out-of frame mutation in the dystrophin messenger can result in in-frame mRNA transcripts that are translated into shorter but functionally active d[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Jamet T ; Daniele N ; Guerchet N ; Tanniou G ; Mandel JL ; Buj Bello A | 2011X-linked Myotubular Myopathy (XLMTM) is the most severe form of centronuclear myopathy, a group of muscular diseases classified together in reason of the presence of abnormally large nuclei localized in central position of hypotrophic myofibres.[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Kunz S ; Bigot A ; Zacharias U ; Mouly V ; Spuler S ; Cartaud J | AFM-TELETHON | 2011Dysferlin gene mutations cause limb-girdle muscular dystrophy (LGMD) 2B and miyoshi myopathy. These muscular dystrophies are characterized by progressive muscle weakness. Dysferlin is known to play an essential role in skeletal muscle membrane r[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Jacobson K ; Petermann O ; Ruegg U | AFM-TELETHON | 2011Duchenne muscular dystrophy is caused by the lack of dystrophin and characterized by membrane fragility and elevated levels of intracellular calcium. This increased calcium concentration aggravates the condition by stimulating proteolysis and ap[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; De Larichaudy J ; Vidal H ; Lefai E ; Nemoz G | AFM-TELETHON | 2011Muscle atrophy associated with a number of systemic diseases such as cancer constitutes a major health problem, due to its contribution to the deterioration of patient status and its impact on mortality. The primary cause of rapid muscle mass lo[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Praud C ; Vauchez K ; Zongo P ; Fiszman M ; Vilquin JT | 2011Murine myoblast transplantation studies in mouse models have provided encouraging results but have underlined important limitations, such as acute cell death and poor migration capacity, beyond the immunological tolerance of the grafted cells. T[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Konig S ; Brawand P ; Bernheim L | AFM-TELETHON | 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)) ; Shelton GD ; Levine J ; Mizisin AP | 2011Canine models of neuromuscular diseases, including those for inherited disorders such as the muscular dystrophies and centronuclear and myotubular myopathies, are playing a critical role in pre-clinical testing of new therapeutic modalities. To [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Bevilacqua JA ; Kleinsteuber K ; Ben Yaou R ; Avaria MDLA ; Ferreiro A ; Demay L ; Chain A ; Richard P ; Urtizberea JA ; Bonne G | 2011Several different human diseases have been linked to mutations in the gene encoding lamin A/C (LMNA). Mutations in LMNA were first associated to autosomal forms of Emery-Dreifuss muscular dystrophy (EDMD), a rare slowly progressive humero-perone[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Erwan G ; Bigot A ; Jollet A ; Butler-Browne GS ; Mouly V ; Furling D | 2011Myotonic Dystrophy type 1 (DM1), one of the most common forms of inherited neuromuscular disorders, is caused by a (CTG)n> 40 repeat expansion in the 3'non-coding region of the DMPK gene. DM1 is an RNA-dominant disorder due to the expression of [...]