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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)) ; Zhou J ; Veillet J ; Tiziano FD ; Servidei S ; Bertini E ; Brahe C ; Durand ME ; Topaloglu H ; Melki J | AFM-TELETHON | 2011Spinal muscular atrophy (SMA) is a clinically and genetically heterogeneous disease characterized by degeneration of lower motor neurons leading to progressive muscle paralysis. The most frequent form is linked to mutations of the SMN1 gene on C[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Voldoire E ; Godet E ; Chabault M ; Leroux S ; Jlali M ; Graulet B ; Pitel F ; Le Bihan Duval E ; Berri C ; Duclos M | AFM-TELETHON | 2011The enzyme beta, beta-carotene-15, 15'-monooxygenase, product of the BCMO-1 gene, is involved in the conversion of carotenoids into retinoids. We recently characterized a polymorphism in the putative promoter of the BCMO-1 gene, associated with [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Nebigil-Desaubry C | 2011Prokineticins are potent angiogenic hormones that use 2 receptors, prokineticin receptor-1 (PKR1) and PKR2, with important therapeutic use in anticancer therapy. Observations of cardiac and renal toxicity in cancer patients treated with antiangi[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Jean-Louis F ; Dubois L ; Boukhatmi H ; deTaffin M ; Crozatier M ; Vincent A | AFM-TELETHON | 2011The diversity of human muscles is necessary to achieve proper coordinated movements. Each muscle displays a distinctive set of properties: size, shape, orientation, attachment sites to the skeleton and specific innervation. The fundamental quest[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Roux-Buisson N ; Santoni E ; Kyndt F ; Albuisson J ; Fauré J ; Lunardi J ; Pavin D ; Mabo P | 2011Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare familial arrhythmogenic disease, characterized by syncope or sudden death induced by emotional or physical stress. The mortality rate in untreated individuals ranges from 30 [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Michon CC ; Nadaj-Pakleza A ; Boneva N ; Payan C ; Gargiulo M ; Herson A ; Guichar-Gomez E ; Berthoux M ; Sarazin M ; Eymard B ; Labrune P ; Laforet P | AFM-TELETHON | 2011BACKGROUND. Glycogen storage disease type III (GSD III) is a rare autosomal recessive disorder due to glycogen debranching enzyme (GDE) deficiency. It results in a multisystemic affection including hepatic and myopathic symptoms. While frequent [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Argov Z | AFM-TELETHON | 2011The clinical and genetic features of hereditary inclusion body myopathy (HIBM)/ distal myopathy with rimmed vacuoles (DMRV), a disorder due to mutations in the Nacetylglucosamine epimerase/N-acetylmannosamine kinase (GNE) gene will be reviewed. [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Strosova M ; Ruegg U ; Dorchies OM | AFM-TELETHON | 2011Green tea polyphenols (GTP) and their major compound epigallocatechin gallate (EGCG) are known for a wide range of health-promoting properties, such as antioxidant, anticancer, anti-inflammatory, antibacterial, antiviral, antifibrotic, hypolipid[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Berardinelli A ; Fagiolari G ; Vallejo D ; Lucchini V ; Bordoni A ; Lamperti C ; Ripolone M ; Corti S ; Balottin U ; Bresolin N ; Comi GP ; Sciacco M ; Moggio M | 2011Spinal muscular atrophy (SMA) is a neuromuscular disorder characterized by degeneration of spinal cord anterior horn presenting with weakness and muscular atrophy. It is caused by mutations in SMN1 gene (Brahe C. 2001) and it is transmitted as a[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Cartaud J ; Cartaud A ; Stetzkowski-Marden F ; Maoui A | 2011Cholesterol/sphingolipid-rich membrane micro-domains or membrane rafts have been implicated in various aspects of receptor function such as receptor activation, trafficking and synaptic localization. More specifically in muscle, membrane rafts a[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Hubers L ; Valderrama-Carvajal H ; Laframboise J ; Sanchez G ; Coté J | AFM-TELETHON | 2011Spinal muscular atrophy is an autosomal recessive neuromuscular disease caused by disruption of the survival of motor neuron gene, SMN, which promotes cytoplasmic assembly of the splicing core machinery. It remains unclear how a deficiency in SM[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Bencze M ; Negroni E ; Yacoub-Youssef H ; Chaouch S ; Wolff A ; Di Santo J ; Chazaud B ; Butler-Browne GS ; Savino W ; Mouly V ; Riederer I | 2011Cell therapy has been envisaged for some genetic muscular disorders using myoblasts in order to deliver the missing protein. Although pre-clinical trials in the mouse were promising, therapeutic trials using local injection of myoblasts failed t[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Le Bihan MC ; Bigot A ; Jensen SS ; Dennis JL ; Rogowska-Wrzesinska A ; Laine J ; Gache V ; Furling D ; Jensen ON ; Voit T ; Mouly V ; Coulton GR ; Butler Browne G | 2011Efficient muscle regeneration requires cross talk between multiple cell types via secreted signalling molecules. However, as yet there has been no comprehensive analysis of this secreted signalling network in order to understand how it regulates[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Laustriat D ; Gide J ; Barrault L ; Furling D ; Peschanski M ; Baghdoyan S | AFM-TELETHON | 2011Myotonic Dystrophy type 1 (DM1), the most common form of inherited muscular dystrophy in adults, is due to an unstable expansion of CTG triplet repeats in the 3'-untranslated region of the DMPK gene. This generates alternate splicing defects in [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Lejeune F ; Gonzalez S | 2011Nonsense mutations are responsible of about 8-10% of Duchenne muscular dystrophies. In most of cases, the consequence is the degradation of the premature termination codon (PTC)-containing mRNA by a mechanism called nonsense-mediated mRNA decay [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Chaouch S ; Mamchaoui K ; Trollet C ; Bigot A ; Negroni E ; Wolff A ; Kandalla P ; Marie S ; Di Santo J ; Muntoni F ; Lacau Saint Guily J ; Spuler S ; Philippi S ; Blumen S ; Furling D ; Voit T ; Wright W ; Aamiri A ; Butler-Browne GS ; Mouly V ; Kandalla PK ; Kim J ; Blumen SC ; Wright WE | AFM-TELETHON | 2011We recently generated immortalized human myoblast cell lines from control donors, by transduction of these cells with both telomerase (hTERT) and cyclin-dependent kinase 4 (CDK-4) expressing vectors. These cells maintain their potential to diffe[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Gwenola M | 2011Almost one fifth of clinical trials of human gene therapy is based on the use of retroviral vectors for gene transfer. Their integration ensures stable transmission of the transgene to progeny cells. However, the provirus may influence host gene[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Bourgeois A ; Duprez D | 2011Skeletal muscle development, growth and regeneration rely on muscle stem cells. An important goal is to understand the nature of the signals regulating these muscle stem cells during myogenesis. The signals regulating the pool of muscle progenit[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Nicot AS ; Pilot-Storck F ; Sandri M ; Schaeffer L ; Goillot E | AFM-TELETHON | 2011Autophagy is essential for cell homeostasis through degradation of proteins and organelles by lysosomes, thus maintaining a balance between synthesis, degradation and recycling. Excessive protein clearance in disused muscles, cancer or some myop[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Whalen S ; Gonzales M ; Laquerrière A ; Quijano Roy S ; Delezoide AL ; Giuliano F ; Richard P ; Le Bail A ; Hainque B ; Chevallier A ; Bieth E ; Avila-Smirnow D ; Heron D ; Sternberg D | 2011IntroductionRecently, neuromuscular junction (NMJ) genes have been implicated in FADS, mainly in multiple pterygium syndromes, lethal (LMPS) or not (Escobar syndrome). Recessive mutations have been identified in CHRNG, RAPSN, CHRND, CHRNA1 and D[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Wary C ; Bolbos R ; Jouvion G ; Baligand C ; Carlier PG | 2011Dynamic in vivo nuclear magnetic resonance (NMR) imaging and spectroscopic investigations of metabolism, perfusion and/or oxygenation has existed in humans for many years, including in interleaved combinations of these1 and serves for clinical i[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Schakman O ; Zanou N ; Shapovalov G ; Gallo C ; Dietrich A ; Lebacq J ; Ruegg U ; Birnbaumer L ; Gailly P | AFM-TELETHON | 2011Skeletal muscle contraction is reputed not to depend on extracellular Ca2+. Indeed, stricto sensu, excitation-contraction coupling does not necessitate entry of Ca2+. However, we previously observed that, during sustained activity (repeated cont[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Hidalgo M ; Launay T ; Buisson N ; Marchand D ; Bigard X ; Beaudry M ; Darribère T | AFM-TELETHON | 2011Oxygen availability plays a central role during early development and normal muscular activity. Hypoxia, a decrease in oxygen availability, seems to induce modifications in the main calcineurin, PI3K and p38-MAPK pathways. To examine the hypoxia[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Iglesias C ; Delestree N ; Manuel M ; Zytnicki D | AFM-TELETHON | 2011In Amyotrophic Lateral Sclerosis (ALS; SOD1 G93A model), only the fast contracting motor units are affected [1,2]. The mechanisms leading to this selective vulnerability are still unknown. In particular, we do not know whether the motoneurons' ([...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Desaphy JF ; Costanza T ; Gramegna G ; Conte Camerino D | AFM-TELETHON | 2011The therapy of myotonia is based on the use of sodium channel blockers able to inhibit the high-frequency discharges of action potentials in skeletal muscle. Mexiletine (Mex) is widely considered as the first choice drug, but a number of myotoni[...]