Détail du congrès:
Congrès: Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) (9-13 mai 2005)
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AFM
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Documents disponibles provenant de ce congrès (412)
Publication AFM
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Braun S ; D'Andon A | 07/2005Texte intégral de l'article : Myologie 2005, Congrès International de Myologie organisé par l'AFM, a permis de faire un point sur les développements thérapeutiques dans les maladies neuromusculaires. Les essais cliniques se multiplien[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Strochlic L ; Cartaud A ; Méjat A ; Grailhe R ; Schaeffer L ; Changeux JP ; Cartaud J | 2005Communication n° 643 The muscle-specific receptor tyrosine kinase (MuSK) is part of an agrinreceptor complex, that orchestrates the differentiation of the neuromuscular junction (NMJ). Activation of MuSK stimulates tyrosine phosphorylation and d[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Araar M ; El Euch G ; Zouari M ; Hentati F | 2005Communication n° 519 Background: Abetalipoproteinemia is a very rare autosomal recessive disease caused by mutations in the MTP gene (Microsomal Triglyceride Transfer Protein). The clinical features include gastrointestinal, ophthalmologic, haem[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Ryten M ; Koshi R ; Knight G ; Greensmith L ; Cockayne D ; Ford A ; Burnstock G | 2005Communication n° 572 The ion-channel subunit P2X2 is one of seven known subunits forming homomeric and heteromeric receptors for ATP. This receptor subunit is expressed in developing mammalian skeletal muscle, but not in adult muscle fibres. The[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Bernard V ; Dobbertin A ; Krejci E | 2005AChE is organized in tetramers by two associated proteins, the collagen Q (ColQ) and a transmembrane protein (PRiMA: Proline Rich Membrane Anchor). ColQ targets tetramers to the basal lamina (BL) and PRiMA to the plasma membrane. Biochemical ana[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Krejci E ; Akaaboune M | 2005Communication n° 342 At the mammalian neuromuscular junction, Acetylcholinesterase (AChE) controls the temporal stimulation of the muscle by the nerve. AChE is concentrated in the basal lamina and classical experiments suggest that this enzyme i[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Hinard V ; Konig S ; Bader C ; Bernheim L | 2005Communication n° 155. During post-natal growth or after muscle injury, skeletal muscle myoblasts differentiate and fuse to form myotubes. We have shown recently that one of the earliest events of the differentiation is the hyperpolarization of h[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Brzoska E. ; Wrobel E ; Moraczewski J | 2005Communication n° 243. Introduction : Cell-extracellular matrix and cell-cell adhesion play essential roles during myogenesis. Several adhesion proteins were postulated to play a role in this process and among them are: integrin a3ß1, M-cadherin,[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Mroue G ; Prigent H ; Orlikowski D ; Bohic M ; Baconnier P ; Lofaso F ; Benchetrit G | 2005Communication n° 662 Patients with advanced neuromuscular diseases often require chronic mechanical ventilation. Long term-assisted ventilation often has to address the problem of leakage, whether the device used is a nasal mask or tracheostomy.[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Nowak K ; Sewry C ; Potter A ; Squire S ; Fisher R ; Marston S ; Davies K ; Laing N | 2005Communication n° 177. INTRODUCTION Mutations in the skeletal muscle alpha-actin gene (ACTA1) have been shown to cause pathologically distinct congenital myopathies: actin myopathy, intranuclear rod myopathy, nemaline myopathy, core-rod myopathy,[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Le Bihan M ; Hou Y ; Coulton G | 2005Communication n° 356. Introduction : Skeletal muscle can hypertrophy, atrophy and alter fibre type in response to external stimuli. Disease also generates typical pathologic changes in phenotype. Identification of molecular networks underpinning[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Riederer I ; Negroni E ; Bencze M ; Savino W ; Mouly V ; Silva-Barbosa SD ; Butler-Browne GS | 2005Communication n° 501 Myoblast transplant therapy (MTT) can be envisioned as a clinical alternative in the treatment of several myopathies. The problems that remain to be solved to improve the efficiency of MTT is the massive early death of trans[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Riederer I ; Negroni El ; Savino W ; Bencze M ; Mouly V ; Silva-Barbosa SD ; Butler-Browne GS | 2005Communication n° 495 Myoblast transplant therapy (MTT) can be envisioned as a clinical alternative in the treatment of several myopathies. The problems that remain to be solved to improve the efficiency of MTT is the massive early death of trans[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Le Panse R ; Clairac G ; Truffault F ; Berrih-Aknin S | 2005Communication n° 506 Myasthenia Gravis (MG) is an autoimmune disease due to autoantibodies directed against components of the neuromuscular junction and leading to disabling fatigability of muscles. Our objective, using microarrays, was to ident[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Bidaud I ; Tuluc P ; Bourinet E ; Nargeot J ; Flucher B ; Lory P | 2005Communication n° 79 Hypokalemic periodic paralysis type 1 (HypoPP1) is associated with mutations within the pore subunit (CaV1.1/a1S) of the skeletal muscle L-type voltage-dependent calcium channel (L-type VDCCs). To date, three missense mutatio[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Jenkins C ; Lebihan-Duval E ; Simon J ; Cogburn LA ; Duclos MJ | 2005Communication n° 713. INTRODUCTION : In order to have a global view of the gene networks controlling growth performance, transcriptome analysis was used on genetic models of chickens divergently selected for high or low growth rate (Ricard et al[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Kordeli E ; Hopitzan A ; Baines A ; Ludosky MA ; Recouvreur M | 2005Communication n° 441. Ankyrins are a family of modular membrane adapter proteins that are ubiquitous in metazoans. They are involved in the biogenesis of membrane domains by coupling diverse cell adhesion molecules (CAMs) and ion channels to the[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Fayet G ; Auré K ; Leroy JP ; Lacene E ; Laforet P ; Ogier de Baulny H ; Romero NB ; Lombès A | 2005Communication n° 381. Mitochondrial DNA (mtDNA) deletions were the first mtDNA alterations identified as the cause of mitochondrial diseases. They have been associated with three clinical presentations : two severe multisystemic diseases with ea[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Ben Yaou R ; Beroud C ; Chikhaoui K ; Helbling-Leclerc A ; Claustres M ; Récan D ; Bonne G | 2005Communication n° 619 Introduction: Laminopathies are a group of 10 heterogeneous disorders caused by mutations of LMNA gene encoding lamins A/C two nuclear envelope proteins. Among laminopathies three disorders specifically affect skeletal and c[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Boerio D ; Lefaucheur JP ; Creange A ; Hogrel JY | 2005Communication n° 234. INTRODUCTION : The achievement of a motor task induces an effort-related fatigability implying several mechanisms. Fatigability can be due to either central (central drive, reflex activity) or peripheral (peripheral nervous[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Chazalette D ; Hnia K ; Rivier F ; Hugon G ; Mornet D | 2005Communication n° 206 Muscle fibers attach to laminin in the basal lamina using two mechanisms that have been studied in this work. The first one involves dystrophin-associated protein complex (DAPC) and the second one implies muscle-specific alp[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; de la Porte S ; Voisin V ; Sébrié C ; Matecki S ; Yu H ; Gillet B ; Ramonatxo M ; Israel M | 2005Communication n° 403 A possible treatment for Duchenne muscular dystrophies would be to compensate for dystrophin loss by increasing the expression of utrophin, another cytoskeletal protein of the muscle membrane. We previously found that L-argi[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Bassez G ; Radvanyi H ; Chapoy E ; Authier FJ ; Eymard B ; Gherardi R | 2005communication n° 536 Introduction : Myotonic dystrophy type 1 (DM1) and type 2 (DM2) are caused by a noncoding (CTG)n and (CCTG)n expansion mutation respectively. Both disorders are characterized by nuclear sequestration of mutant CUG-containing[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Cossee M ; de Saint-Martin A ; Samimi S ; Recan-Budiartha D ; Laugel V ; Mohr M ; Mandel JL ; Koenig M | 2005Communication n° 544 Duchenne and the less frequent Becker muscular dystrophy (DMD/BMD) are X-linked recessive disorders caused by mutations in the gene encoding the dystrophin muscle skeletal protein. In contrast with the severe DMD form in whi[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Salloum A ; Massard A ; Cuisset JM ; Hurtevent JF ; Vallée L | 2005Communication n° 360 SUMMARY: Acute motor axonal neuropathies (AMAN) have been recently individualised as a specific entity in patients presenting with Guillain Barré syndrome. In the literature, very few pediatric cases have been so far describ[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Laugraud A ; Ségalat L | 2005Communication n° 58. We study muscle degeneration due to the absence of dystrophin. This protein and other molecules of the dystrophin complex have conserved homologues in the nematode Caenorhabditis elegans genome. The structural and molecular [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; van Parijs V ; van Goethem G ; Martin J ; van den Bergh P | 2005Communication n° 317. Autosomal dominant progressive external opththalmoplegia (adPEO) is characterized by ptosis and weakness of the extraocular muscles. Mutations within several nuclear genes (ANT1, C10orf2, POLG) were recently discovered to b[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Navarro C ; Sobrido J ; Fernandez J ; Fontoira E ; Perez-Sousa C ; Cabello A ; Teijeira S ; Rivas E ; Delague V | 2005Communication n° 352. INTRODUCTION : Congenital Fibre Type Disproportion (CFTD) is a non- or slowly progressive muscle disease with relative type 1 fibre smallness. The natural course is variable and a severe progression has been observed in som[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Viollet L ; Maystadt I ; Leclair-Richard D ; Estournet Mathiaud B ; Barois A ; Renault F ; Routon MC ; Durand MC ; Lefebvre S ; Munnich A ; Verellen-Dumoulin C | 2005Communication n° 577 Lower Motor Neuron diseases (LMND) form a wide spectrum of rare sporadic and hereditary neuromuscular disorders, characterized by progressive degeneration of the motor neurons of the spinal cord. We reported here the clinica[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Serose A ; Prudhon B ; Salmon A ; Doyennette-Moyne MA ; Fiszman M ; Fromes Y ; Doyennette MA | 2005Communication n° 202 Introduction : As cardiac disorders are a common complication in neuromuscular disorders, it seems essential to treat both skeletal and cardiac muscles in these pathologies. Dilated cardiomyopathies (DCM) are due to progress[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Le Rumeur E ; Cluchague N ; Rocher C ; Pottier S ; Cherel Y | 2005Communication n° 86. Duchenne Muscular Dystrophy (DMD), and mdx mouse and grmd dog dystrophies result from X-linked genetic defects leading to a lack of dystrophin. Dystrophin is involved in the dystrophin-glycoprotein complex (DGC). Until now, [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Benabdallah BF ; Bouchentouf M ; Tremblay J | 2005Communication n° 170. Background : Duchenne muscular dystrophy is caused by a dystrophin gene mutation. Transplantation of normal myoblasts results in long-term restoration of dystrophin. However, the success of this approach is compromised by t[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Salah N ; Millet G ; André-Schmutz I ; Desforges B ; Olaso R ; Roblot N ; Courageot S ; Bensimon G ; Cavazzana-Calvo M ; Melki J | 2005Communication n° 174 INTRODUCTION : Several studies have demonstrated that adult bone marrow-derived cells (BMDC) can participate in skeletal muscle regeneration after bone marrow transplantation (BMT). However, the mechanisms and identities of [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Gherardi RK ; Chrétien F ; Caramelle P ; Christov C ; Dreyfus P | 2005Communication n° 321 We have previously developed a murine model of bone marrow (BM) transplantation from B6-TgGFP transgenic mice to normal irradiated B6 mice, the cytoplasmic green fluorescent protein (GFP) being used as an unambiguous marker [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Sikiric P ; Staresinic M ; Novinscak T ; Batelja L ; Brcic L ; Jukic I ; Pevec D ; Kokic N ; Anic T ; Buljat G ; Zoricic I ; Perovic D ; Seiwerth S | 2005Communication n° 547. INTRODUCTION : Stable gastric pentadecapeptide BPC 157 (GEPPPGKPADDAGLV, M.W. 1419 (PL-10, PLD-116, PL 14736 Pliva, Croatia)), accelerates healing of wounds, burns as well as transected Achilles tendon and shows strong anti[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Lafreniere JF ; Mills PL ; Skuk D ; Tremblay J | 2005Communication n° 302 Introduction: One problem for the clinical application of myoblast transplantation for Duchenne Muscular Dystrophy (DMD) treatment is the poor migration of transplanted cells. Recently, we described a new approach that consi[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Pongs O ; Schule-Bahr E ; Choe C ; Neu A ; Isbrandt D ; Schulze-Bahr E | 2005Communication n° 8 Beta-adrenergic receptor-mediated, cAMP, and protein kinase A-dependent (PKA) stimulation of select cardiac ionic currents, such as the rapid outward potassium current (IKr), controls ventricular action potential duration at h[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Mège RM ; Marthiens V ; Boscher C ; Gavard J ; Lambert M | 2005Communication n° 252. We previously observed that cadherin-11, a type II cadherin, is expressed in growing motor and sensory axons in the mouse embryo. Here, we assessed its functional involvement in the regulation of axon elongation and fascicu[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Laporte J ; Nicot AS ; Fares H ; Chisholm DJ ; Mandel JL ; Labouesse M | 2005Communication n° 186. Myotubular myopathy is an X-linked congenital disorder which results, in affected new-born males, in a very severe and generalized muscle weakness. The gene mutated in patients, MTM1, encodes a 3-phosphoinositides (PtdIns) [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Chakkalakal J ; Jasmin B | 2005Communication n° 421. Introduction : We have previously shown that slow/oxidative muscle fibers contain significantly more utrophin in comparison to fast/glycolytic fibers (Gramolini et al., 2001). More recent studies have implicated calcineurin[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Mazères G ; Leloup L ; Cottin P ; Brustis JJ | 2005Communication n° 443. Introduction : The calcium dependent proteolytic system, composed of ubiquitous and tissue specific calpains, is involved in many physiological events such as cell cycle, muscle cell differentiation, cell spreading and moti[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Arnaudeau S ; Holzer N ; Konig S ; Bader CR ; Bernheim L | 2005Communication n° 137. Myoblast differentiation is a strictly Ca2+-dependent process. We have shown that a main route for Ca2+ entry is through T-type Ca2+ channels. However, because of the importance of cytoplasmic Ca2+ for differentiation, myob[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Courdier-Fruh I ; Magyar JP ; Briguet A | 2005Communication n° 27 Previous studies in mdx mice indicate that upregulation of the cytoskeletal protein utrophin, a structural and functional homologue of dystrophin, offers a potential treatment strategy for Duchenne muscular dystrophy (DMD). C[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Goudenege S ; Poussard S ; Cottin P | 2005Communication n° 365. Introduction : We have previously shown that calpain promotes myoblast fusion by acting on protein kinase Ca and MARCKS (Myristoylated Alanine Rich C kinase Substrate), a protein involved in actin cytoskeleton dynamic and m[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Skuk D ; Goulet M ; Roy B ; Tremblay J | 2005Communication n° 139 Introduction : Our Myoblast Allotransplantation Studies In Monkeys Showed That The Absence Of Immunosuppression Leads To Graft Rejection In A Few Days, While A Good Immunosuppression Allows Graft Survival Apparently As Long [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Ben Yaou R ; Meune C ; Bécane HM ; van Berlo JH ; Anselme F ; Eymard B ; Hannequin D ; Urtizberea JA ; Pinto YM ; Bonne G ; Duboc D | 2005Communication n° 566 Introduction : Patients with lamin A/C gene (LMNA) mutations have frequent cardiac involvement characterized by conduction defects and arrhythmias, heart failure and high frequency of sudden death (SD). While SD is not adequ[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Carrier L ; Sarikas A ; Schenke C ; Doll D ; Flavigny J ; Lindenberg K ; Eschenhagen T ; Zolk O ; Lindenberg KS | 2005Communication n° 280 Cardiac myosin binding protein C (cMyBP-C) gene mutations are a frequent cause of familial hypertrophic cardiomyopathy (FHC), and most of them result in C-terminal truncated cMyBP-Cs. However, truncated cMyBP-Cs were undetec[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Pons B ; Fraley A ; Zuber G ; Behr JP | 2005Communication n° 97 The basic concept behind gene therapy is to transfer a gene into the cell nucleus and thereby express a missing protein. An alternative to treat inherited and acquired genetic disorders is the use of small oligodeoxyribonucle[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Gartioux C ; Allamand V ; Maugenre S ; Ledeuil C ; Sabatelli P ; Quijano Roy S ; Estournet Mathiaud B ; Richard P ; Pepe G ; Romero NB ; Guicheney P | 2005Communication n° 408 Ullrich congenital muscular dystrophy (UCMD) belongs to the subgroup of merosin-positive congenital muscular dystrophies. Early signs may be neonatal, such as arthrogryposis, torticollis, hip dislocation. It is characterized[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Nordez A ; Cornu C | 2005Communication n° 626. Muscle stretching exercises are used for functional rehabilitation and to limit the decrease in joint flexibility induced by neuro-muscular pathologies. Nevertheless, effects of stretching are discussed and seem to depend o[...]