Détail du congrès:
Congrès: Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) (9-13 mai 2005)
Commentaire :
AFM
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Documents disponibles provenant de ce congrès (412)
Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Tüzün E ; Saini S ; Yang H ; Higgs S ; Christadoss P | 2005Communication n° 5 Introduction: The classical complement pathway (CP) is involved in the induction of experimental autoimmune myasthenia gravis (EAMG) induced by acetylcholine receptor (AChR) immunization. Objective: To delineate the significan[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Teissié J ; Golzio M ; Franchi R | 2005Communication n° 308. Expression of fluorescent proteins (GFP, DsRed) after gene transfer can be conveniently followed in living animals (mouse) directly by fluorescence video imaging. We routinely used this approach for a relative quantitative [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Shafeghati Y ; Martin GM | 2005Communication n° 3 Abstract Two Iranian cases with very rare progeroid syndromes are reported. The first was a 24-year-old young girl. She was healthy till her 13th birthday. From that time she has been suffering gradually from a progressive gen[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Kuntzer T ; Marin C ; Pahnke J ; Benhattar J ; Janzer RC | 2005Communication n° 265 Muscle biopsy in polymyositis patients is not considered to be of prognostic help. So far, it is not able to predict the response to steroids, nor does it reflect the clinical outcome. Previous findings indicate that in a nu[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Adamy C ; Arimura T ; Candiani G ; Decostre V ; Varnous S ; Massart C ; Pecker F ; Bonne G | 2005Communication n° 279 Emery-Dreifuss muscular dystrophy (EDMD) is characterized by early joint contractures, slowly progressive muscular wasting and weakness, and by adult age, development of cardiac conduction defects, arrhythmias, left ventricu[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Taratuto AL ; Saccoliti M ; Lubieniecki F ; Panigazzi A ; Castano JG ; Romero NB | 2005Communication n° 50. Congenital myopathies are characterized by the presence of distinctive structural and histochemical diagnostic features with type 1 predominance and/or hypotrophy but diagnosis may be inconclusive at muscle biopsy during ear[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Herlicoviez D ; Rolland M ; Chapon F ; Berthelin C ; Fromager G ; Dress D ; Viader F | 2005Communication n° 48. Muscle-specific phosphorylase b kinase (PbK) deficiency is an unusual form of glycogen storage disorder. PbK converts the inactive form of glycogen phosphorylase into its active form. Most patients are male, complain of exer[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Bassez G ; Andreux F ; Laforet P ; Radvanyi H ; Jeanpierre M ; Eymard B | 2005Communication n° 538 Introduction: myotonic dystrophy type 1 (DM1) typically involves distal limb muscles at onset whereas a proximal muscle involvement is more suggestive of myotonic dystrophy type 2 (DM2). In DM1, proximal musculature, includi[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Bassaglia Y ; Cebrian J ; Covan S ; Foucrier J | 2005Communication n° 490. Proteasomes are the major actors of non-lysosomal cytoplasmic protein degradation. In particular, these large protein complexes (about 2500 kDa) are considered to be responsible for muscular degradation during skeletal musc[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Héron-Milhavet L ; Franckhauser C ; Rochat A ; Hemmings B ; Fernandez A ; Lamb N | 2005Communication n° 639. PKB/Akt kinases are involved in signaling downstream of growth factor receptor tyrosine kinases and phosphatidylinositol 3-kinase. Akt1 and Akt2 are the two principle Akt isoforms expressed in all tissues. We have used smal[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Le Bihan M ; Bigot A ; Butler-Browne GS ; Mouly V ; Furling D ; Coulton G | 2005Communication n° 571. Introduction : The proliferation of human satellite cells in vitro is limited by replicative senescence. For example, satellite cells isolated from newborns can achieve 55-60 divisions in vitro, while cells isolated from ad[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Roccella M ; Parisi L ; Canziani T | 2005Communication n° 111 Purpose : Dystrophinopathy is the first cause of myopathy in children, and Duchenne muscular dystrophy (DMD) is one of the most severe and frequent forms of this disease. In the last years, even if medical cures allowed thes[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Haddad H ; Richard P ; Gaudon K ; Ioos C ; Barois A ; Boespflug-Tanguy O ; Mayer M ; Bauche S ; Koenig J ; Hantaï D ; Eymard B | 2005Communication n° 699 Background: Congenital myasthenic syndromes (CMS) are a heterogeneous group of hereditary disorders due to defects in the neuromuscular transmission. According to the site of the primary defect, these syndromes are classifie[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Martinez-Martinez P ; Losen M ; Van de Waarenburg M ; Machiels BM | 2005Communication n° 569 The primary autoantigen in myasthenia gravis, the acetylcholine receptor (AChR), is clustered and anchored in the postsynaptic membrane of the NMJ by rapsyn. Previously, we found that the overexpression of rapsyn by in vivo [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Heron D ; Cohen D ; Plaza M ; Angeard N ; Lanthier O ; Eymard B ; Guile JM | 2005Communication n° 436 This study investigated reading and spelling difficulties in subjects with the juvenile form of myotonic dystrophy (MD). Twenty three consecutive patients with juvenile MD who were referred to a special clinic were assessed [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Bouchentouf M ; Benabdallah BF ; Dumont M ; Tremblay J | 2005Communication n° 39. BACKGROUND : The quantification of the graft success is a key element to evaluate the efficiency of cellular therapies for several pathologies such as Duchenne muscular dystrophy. This study describes a novel approach to eva[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Boerio D ; Hogrel JY ; Creange A ; Lefaucheur JP | 2005Communication n° 231. INTRODUCTION : Following an action potential, peripheral nerve axons become first totally unexcitable: absolute refractory period (ARP) then partially unexcitable: relative refractory period (RRP). OBJECTIVE : To compare va[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Athanasopoulos T ; Foster H ; Graham I ; Hill V ; Perez N ; Vulin A ; Fabb SA ; Garcia L ; Danos O ; Dickson G | 2005Communication n° 635 Introduction : Duchenne muscular dystrophy (DMD) is a lethal genetic muscle disorder affecting 1:3500 male individuals, caused by recessive mutations in the dystrophin gene. The size of the gene (2.4Mb) and mRNA (14kb) in ad[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Losen M ; Martinez-Martinez P ; Machiels BM | 2005Communication n° 423 Various mutants of the human anti-human-acetylcholine receptor (AChR) antibody IgG1-637 were produced and characterised. A monovalent version of this AChR antibody was created by deleting four amino acids in the CDR3 of one [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Scamps F ; Hilaire C ; Al-Jumaily M ; Inquimbert P ; Valmier J | 2005Communication n° 47 Unsuccessful regeneration of sensory neurons following nerve injury leads not only to ataxia but is also associated to persistent spontaneous electrical activity that plays an important role in the occurrence and maintenance [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Arbogast S ; Reid MB | 2005Communication n° 309 Under physiological conditions, muscle fibers continually produce reactive oxygen species (ROS) and nitric oxide (NO). Intracellular oxidant activity is increased by fatiguing exercise, inflammatory mediators, and heat stres[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Rana-Poussine V ; L'Honoré A ; Héron-Milhavet L ; Franckhauser C ; Lamb N ; Fernandez A | 2005Communication n° 645. Skeletal muscle comprises two principal cell forms, myofibres executing muscle contraction and satellite cells, responsible for muscle growth and regeneration. Three stages can be identified in muscle development : muscle f[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Melms AR ; Luther C ; Poeschel S ; Varga M ; Brucklacher V ; Melms A ; Tolosa E | 2005Communication n° 576 Thymus-derived CD4+CD25hi T regulatory cells play a decisive role in the generation and maintenance of peripheral tolerance. These cells are naturally hyporesponsive in vitro and can inhibit the proliferation of co-cultured [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Gorni K ; Zhao P ; Pasquali L ; Hoffman E | 2005Communication n° 164. Introduction : The absence of dystrophin protein leads to different phenotypes in different species, despite complete loss of dystrophin in all muscles. Patients affected by Duchenne Muscular Dystrophy show chronic degenera[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Mairet S ; Maïsetti O ; Portero P | 2005Communication n° 600. Introduction. Muscular architecture, characterized by fascicle length (FL), pennation angle (Pa), and muscle thickness (MT), can be studied in vivo by using ultrasonography. The parameters are determinant for muscle force e[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Pitard B ; Bossard F | 2005Communication n° 94 A variety of muscular disorders including Duchenne muscular dystrophy are potentially amenable to a treatment modality in which a therapeutic nucleic acids is directly delivered to the muscle. We recently reported that nonion[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Laforet P ; Escolar D ; Pestronk A ; Jaffe K ; Carter J ; Berger K ; Rapoport D ; Skrinar AM ; Doppler V ; Eymard B ; Wokke J | 2005Communication n° 470. Background : Pompe disease is a rare, autosomal recessive, metabolic muscle disease caused by a deficiency of lysosomal acid ?-glucosidase (GAA), an enzyme that degrades glycogen. There is a broad spectrum of disease rangin[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Bonnieu A ; Hamade A ; Deries M ; Bally-Cuif L ; Begemann G ; Genet C ; Sabatier F ; Cousin X | 2005Communication n° 226. Retinoic acid (RA) is involved in the control of a variety of differentiation processes. In vitro experiments have clearly shown that RA blocks myoblast proliferation and induces their differentiation. We are studying RA fu[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Haddad H ; Leturcq F ; Romero NB ; Caizergues D ; Benveniste O ; Veres G ; Chaouch M ; Kaplan JC ; Herson S ; Urtizberea JA | 2005Communication n° 698 Background : Limb girdle muscular dystrophy type 2C (LGMD 2C) is a rare autosomal recessive muscular disorder caused by mutations in the gamma sarcoglycan (?-SG) gene. Patients with LGMD 2C frequently present with proximal a[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Allamand V ; Richard P ; Desjardin D ; Ferreiro A ; Gartioux C ; Pellegrini N ; Urtizberea JA ; Guicheney P | 2005Communication n° 607 Mutations in the coding sequence of the SEPN1 gene encoding the selenoprotein N (SelN) are responsible for Rigid Spine Muscular Dystrophy (RSMD1), multiminicore disease (MmD) and desmin-related myopathy with Mallory Body-lik[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Coté J ; Tadesse H ; Paquette B | 2005Communication n° 6 Autosomal recessive spinal muscular atrophy (SMA) is a disease that is amongst the leading genetic cause of infants death. SMA is caused by disruption of the "survival of motor neurons" gene (Smn1). SMN plays an essential role[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Vaillend C ; Rosenvald Y ; Fort P ; Candelario A ; Mornet D ; Guégan M ; Martinez-Rojas D ; Rendon A ; Nudel U ; Yaffe D ; Laroche S | 2005Communication n° 620 Mutations affecting Dp71, major product of the dystrophin gene in the brain, are thought to be responsible for the most severe cases of mental retardation in DMD. To investigate this hypothesis, we evaluated the biochemical [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Goutebroze L ; Cifuentes-Diaz C ; Irinopoulou T ; Carnaud M ; Giovannini M ; Girault JA | 2005Communication n° 496. Myelination allows the fast propagation of action potentials at a low energetic cost. It provides an insulating myelin sheath, regularly interrupted at the nodes of Ranvier where are concentrated voltage-gated Na+ channels [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Carré-Pierrat M ; Chambonnier L ; Ségalat L | 2005Communication n° 56. Our group investigates the molecular mechanisms of Duchenne Muscular Dystrophy (DMD) in the nematode Caenorhabditis elegans and in mdx-5Cv mice. In the years 2002 and 2003, we performed a random screen for compounds able to [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Charnet P ; Rousset M ; Kajava A ; Cens T | 2005Communication n° 185. Voltage-gated calcium channels are highly selective for divalent cations. The locus of this selectivity has been ascribed to a ring of negatively charged glutamate (the EEEE locus) highly conserved among high-voltage activa[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Rafai M ; Bourezgui M ; Sibai M ; Gam I ; El Moutawakil B ; Boulaajaj FZ ; Slassi I | 2005Communication n° 617 Mitochondrial cytopathies represent very heterogeneous a group of affections related to a dysfunction of the respiratory chain of mitochondrion. Their expression is polymorphic and plurisystemic. The progressive external oph[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Gonzalez V ; Quijano Roy S ; Parain K ; The ENMC CMD Consortium ; The MMD ENMC Consortium Reseau Dystrophies Musculaires Congénitales ; Leroy JP ; Kaindl A ; Lochmuller H ; Bonnemann C ; Fidzianska A ; Estournet Mathiaud B ; Richard P ; Guicheney P ; Ferreiro A | 2005Communication n° 523. Recently, we found that mutations of the SEPN1 gene, encoding the novel selenoprotein N, cause 3 early-onset myopathies: Rigid Spine Muscular Dystrophy (RSMD1), classical multiminicore disease (MmD) and desmin-related myopa[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Cote F ; Fligny C ; Fromes Y ; Bayard E ; Mallet J ; Vodjdani G | 2005Communication n° 500. Serotonin (5-HT) controls multiple biological functions. While its importance in the nervous system is appreciated, its role at the periphery remains poorly understood. The rate-limiting step in 5-HT biosynthesis, is cataly[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Bacou F ; Coulet B ; Lacombe F ; Lazerges C ; Daussin PA ; Micallef JP ; Chammas M ; Reyne Y | 2005Communication n° 70 Skeletal muscle demonstrates a force deficit after repair of injured peripheral nerves. We tested the hypothesis that the properties of reinnervated rabbit tibialis anterior (TA) muscles improves after transfer of adult myobl[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Sirvent P ; Lacampagne A ; Vassort G ; Raynaud E ; Bordenave S ; Vermaelen M ; Mercier J | 2005Communication n° 570. Statin drugs represent the major improvement in the treatment of hypercholesterolemia. Beneficial effects of these drugs are numerous. Nevertheless, statins present side effects on muscle function characterized by myalgia, [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Kothary R ; de Repentigny Y ; Shafey D ; Côté P ; Bowerman M ; Pinheiro B | 2005Introduction : Spinal muscular atrophy (SMA) is a neurodegenerative disorder characterized by the degeneration of the alpha-motor neurons in the spinal cord. The loss of these neurons causes proximal, symmetrical limb and trunk muscle weakness t[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Chrétien F ; Brigitte M ; Plonquet A ; Chazaud B ; Gherardi RK | 2005Communication n° 373 The role of macrophages and dendritic cells in the pathophysiology of inflammatory myopathies has been recently enlighted. As observed in some patients with dermatomyositis (DM) and in a DM-like condition, likely associated [...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Fergani A ; Dupuis L ; Gonzales De Aguilar J ; Oudart H ; Loeffler JP | 2005Communication n° 710 Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease characterized by selective loss of motor neurons and progressive muscle wasting. We have recently shown that mutant Cu/Zn-superoxide dismutase t[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Heron D ; Jacquette A ; Angeard N ; Devillers M ; Eymard B ; Quera-Salva MA | 2005Communication n° 543 MD type 1 is an autosomal dominant trinucleotide repeat disorder that shows anticipation. Several forms exist which differ in age of onset and severity. The most common is the classical adult form with muscular and polysyste[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Lapucci C ; Pomarè Montin D ; Pandolfo M | 2005Communication n° 531 INTRODUCTION: autosomal recessive spinal muscular atrophy (SMA) is clinically classified in type I (severe), II (intermediate), III (mild). SMA is caused by a loss of function of the Survival of Motor Neuron (SMN) protein. T[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Meier T ; Briguet A ; Coudier-Fruh I ; Foster M ; Erb M ; Lescop C ; Siendt H ; Herzner H ; Henneboehle M ; Weyermann P ; von Sprecher A ; Magyar J | 2005Communication n° 28 Calpain I and II are ubiquitous calcium-dependent cytosolic cystein proteases that cleave intracellular substrates such as cytoskeletal and myofibrillar proteins. Activation of calpains in dystrophin-deficient muscle cells wi[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Gailly P ; Ducret T ; Vandebrouck C | 2005Communication n° 618 In skeletal muscle, Ca2+ is implicated in contraction and in regulation of gene expression. An alteration of [Ca2+]i homeostasis is responsible, at least partially, of the muscle degeneration that occurs after eccentric cont[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Beaulieu D ; Furling D ; Puymirat J | 2005Communication n° 120. Myotonic dystrophy (DM1), the most common form of inherited neuromuscular disease, is caused by a CTG repeat expansion at chromosome 19q23. Fetal muscle development is affected in patients with a congenital form of the dise[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Hu Y ; Eymard B ; Laforet P ; Heron D ; Leturcq F ; Llense S ; Chelly J ; Romero NB ; Logeart D | 2005Communication n° 714 A 50-year-old man without any family history presented a dilated cardiomyopathy, at the age of 41, discovered in the etiologic inquiry of an ischaemic cerebral stroke. From then on, he has suffered moderate asymmetric muscle[...]Article
Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ; Marionneau C ; Couette B ; Liu J ; Li H ; Mangoni M ; Nargeot J ; Lei M ; Escande D ; Demolombe S | 2005Communication n° 687 Even though sequencing of the mammalian genome has led to the discovery of a large number of ionic channel genes, identification of the molecular determinants of cellular electrical properties in different regions of the hea[...]