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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)) ; Trollet C ; Anvar S ; Venema A ; Hargreaves I ; Foster K ; Vignaud A ; Ferry A ; Negroni E ; Gidaro T ; Hourde C ; Baraibar M ; Hoen P ; Davies J ; Rubinsztein D ; Heales S ; Mouly V ; van der Maarel S ; Raz V ; Butler Browne G ; Dickson G | AFM-TELETHON | 2011Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset disorder characterized by ptosis, dysphagia and proximal limb weakness. Autosomal dominant OPMD is caused by a short (GCG)8-13 expansions within the first exon of the poly(A) binding pr[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Karmouch J ; Sigoillot S ; Bourgeois F ; Krejci E ; Chevalier C ; Houlgatte R ; Leger J ; Legay C | 2011Congenital myasthenic syndromes (CMS) correspond to a class of human pathologies resulting from mutations in genes expressed at the neuromuscular junction (NMJ). These disorders are characterized by a dysfunction of the NMJ leading to muscle wea[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Huguet A ; Medja F ; Nicole A ; Vignaud A ; Ferry A ; Guiraud-Dogan C ; Mousiel E ; Metzger F ; Sebille A ; Gomes Pereira M ; Puymirat J ; Bassez G ; Furling D ; Gourdon G | AFM-TELETHON | 2011Myotonic dystrophy type I (DM1) is a dominant disease, highly variable and associated with multisystemic symptoms. The adult onset form presents muscle weakness, myotonia, cardio-respiratory problems, cataracts, hypersomnia, hyperinsulinism, tes[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Delalande O ; Del Giudice E ; Hubert JF ; Le Rumeur E | 2011Dystrophin is a long filamentous protein, its central region is made by 24 successive spectrin-like repeats structured in a coiled-coil (bundle of three alpha-helices) arrangement [1]. Its biological function is essential for keeping the integri[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Segura L ; Escobar Cedillo RE | AFM-TELETHON | 2011Introduction: The motor sensory neuropathies Charcot Marie Tooth (CMT) can be classify genetically as type 1, 2 and x link chromosome, later can be divided in subtypes A, B and C based on the genotype. CMT is the most common hereditary neuropath[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Karine C ; Daniele N ; Florence L ; Vihola A ; Roudaut C ; Monjaret F ; Pion D ; Tarrade A ; Sarparanta J ; Udd B ; Richard I | 2011Titin is a giant protein expressed in both skeletal and cardiac muscles. Several pathogenic mutations were identified in its two last exons causing muscular dystrophy phenotypes. The most common mutation, FINmaj, results in the replacement of 4 [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Bertrand A ; Renou L ; Papadopoulos A ; Beuvin M ; Lacene E ; Arimura T ; Gruenbaum Y ; Bonne G | 2011LMNA gene encodes for lamins A/C, ubiquitous proteins of the nuclear envelope in post-mitotic cells. Lamin A/C are thought to have structural but also essential regulatory roles in various signalization pathways by interactions with transcriptio[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Rome S ; Forterre A ; Chikh K ; Jalabert A ; Pesenti S ; Euthine V ; Nemoz G ; Lefai E ; Vidal H | AFM-TELETHON | 2011Exosomes are nanovesicles of endocytic origin secreted by different cell types including epithelial, hematopoietic, and some tumor cells. They are also present in some biological fluids such as serum, urine, breast milk, and bronchoalveolar lava[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Sdika M ; Tonson A ; Cozzone PJ ; Bendahan D | AFM-TELETHON | 2011Introduction: Accurate muscle size quantification is essential to investigate muscle function related to exercise adaptation, ageing and neuromuscular pathologies. Currently, MRI muscle volume quantification is the gold standard; however this te[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Rosa A ; Corona ED ; Jacquelin D | AFM-TELETHON | 2011MULTIPLE PROTEIN DOMAINS CONTRIBUTE TO NUCLEAR ENTRANCE OF DUX4 E. Daniel Corona, Daniela Jacquelin and Alberto L. RosaLaboratorio de Biologelular y Molecular, Fundacilende, Hipo Irigoyen 384, Cba, Argentina. Twelve years ago it was hypothesized[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Reedy A ; Gieseler K | 2011Proteotoxicity, as defined by cellular pathologies associated with detrimental protein products, has been well defined in neurodegenerative disease such as Alzheimer's, Amyotrophic lateral sclerosis (ALS), Huntington and Parkinson's diseases. Th[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; van den Berg L ; Drost M ; Schaart G ; de Laat J ; van Doorn P ; van der Ploeg A ; Reuser A | 2011Background: Pompe disease is an inherited lysosomal glycogen storage disorder caused by the deficiency of acid a-glucosidase. Skeletal muscle damage is attributed to lysosomal rupture and the release of glycogen and lysosomal enzymes into the cy[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Brigite M ; Adriouch S ; Authier FJ ; Boyer O ; Gherardi RK ; Chrétien F | 2011Myology textbooks describe muscle fibroblasts on the grounds of EM studies as independent cells and envisage them only as collagen-producing cells. Lack of muscle fibroblast marker likely represents one cause of our ignorance about this cell sub[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Chen SF | 2011Purpose: To study the ultrastructural changes of muscle in patients with cerebrotendinous xanthomatosis (CTX) and make a correlation with the Tc99m-sestamibi thighSPECT/CT images (a noninvasive tool for the evaluation of the mitochondrial status[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Trollet C ; Perie S ; Gidaro T ; Mamchaoui K ; Negroni E ; Bouazza B ; Svinartchouk F ; Blumen S ; Mouly V ; Lacau Saint Guily J ; Butler Browne G | AFM-TELETHON | 2011Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant inherited, slow progressing, late onset degenerative muscle disorder, characterized by progressive eyelid drooping (ptosis) and difficulties with swallowing (dysphagia). The phar[...]Article
Muscle proteins and cellular dynamics during somitogenesis and early myogenesis in zebrafish embryos
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Ruf-Zamojski F ; Trinh L ; Megason S ; Trivedi V ; Fraser SE | AFM-TELETHON | 2011High-resolution cellular and molecular data from developing organisms has driven many of the advances in developmental biology. These same approaches, applied to muscle biology, should enable a better understanding of the key events of dynamics [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Feferman T ; Sylvain B ; Mélinée C ; Mouly V ; Tzartos S ; Fuchs S ; Souroujon M ; Berrih-Aknin S | AFM-TELETHON | 2011Anti-acetylcholine receptor (AChR) autoantibodies target the muscle in human spontaneous MG and its induced model experimental autoimmune MG (EAMG). The aim of the study was to identify the common and specific molecular signatures of the muscle [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Del Fraro G ; Meregalli M ; Farini A ; Belicchi M ; Parolini D ; Razini P ; Cassinelli L ; Angeloni V ; Maciotta S ; Bresolin N ; de Silva Bizario J ; Garcia L ; Torrente Y | 2011In DMD, skeletal and cardiac muscles are affected, leading to wheelchair dependency, respiratory failure and premature death. A combination of different strategies might enhance the possibility of successful therapy. We isolated CD133+ cells fro[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Bonnamy B ; Barrey E ; Mata X ; Chaffaux S ; Guerin G | 2011MicroRNAs (miRNA) are small endogenous non-coding interfering RNA molecules (18-25 nucleotides) regarded as major regulators in eukaryotic gene expression. They play a role in developmental timing, cellular differentiation, signaling and apoptos[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Rafai MA ; Manaf S ; Raji L ; Oumari S ; Boulaajaj F ; Ridai M ; El Moutawakil B ; Slassi I | AFM-TELETHON | 2011Introduction : Epidemiological data showed that 10-20% of myasthenic patients have a thymoma. Object: to report cases of myasthenia gravis revealing invasive thymoma, and to specify the particularities of the association myasthenia gravis-invasi[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Vohanka S ; Bednarik J ; Fajkusova L ; Sedlackova J | AFM-TELETHON | 2011Myotonic dystrophy type 1 (MD1) is an autosomal-dominant multisystemic disorder linked to a monoallelic expansion of the CTG n repeat in the 3 untranslated region of the DM protein kinase (DMPK) gene. Healthy individuals have repeats of n= 5-37,[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Vasseur S ; Mauda C | AFM-TELETHON | 2011Myobank-AFM at the Institut de Myologie: human tissue samples and byproducts at the disposal of the scientists in the field of neuromuscular diseases.Thomas Voit Maud Chapart Stane VasseurThe AFM tissue bank for research was created in 1996. It [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Bel C ; Bidault des Chaumes A ; Mahaut I ; Orssaud L | 2011What is Myobase ?Myobase is a bilingual database (French/English) which provides access to bibliographic information updated daily in the field of myology and motor handicap. It is implemented by the AFM Library. What would you find in Myobase ?[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Lambert V ; Capderou A ; Gouadon E ; Le Bret E ; Rucker-Martin C ; Dinanian S ; Stos B ; Renaud JF | 2011Purpose: Advances in cardiac surgery have improved short-term prognoses of patients with congenital heart diseases but, at long-term, right ventricular (RV) failure may occur, leading to morbidity and mortality. As conventional therapy gives poo[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Santosh V | 2011Myofibrillar myopathies(MFM) are uncommon, clinically and genetically heterogenous conditions morphologically characterized by myofibrillar structural changes. six cases of MFM diagnosed between 2003-2010 basing on light and electron microscopic[...]