Détail du congrès:
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)
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[...]Article
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 [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Vezain M ; Gérard B ; Drunat S ; Funalot B ; Fehrenbach S ; N’Guyen-Viet V ; Vallat JM ; Frebourg T ; Tosi M ; Martins A ; SaugierVeber P | AFM-TELETHON | 2011Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder characterized by degeneration of alpha motor neurons in the anterior horn cells of the spinal cord, often appearing during early childhood. This disease results in mo[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Gicquel Zouida E ; Karine C ; Daniele N ; Richard I | AFM-TELETHON | 2011Evelyne Gicquel, Karine Charton, Nathalie Dani and Isabelle Richard.Gthon, 1 bis rue de l'Internationale, 91000 Evry, France.Limb Girdle Muscular Dystrophies (LGMD) constitute a group of myopathies affecting muscles of shoulder and pelvic girdle[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Sharma MC ; Pathak P ; Kaushal S ; Sarkar C ; Suri V ; Mohd H ; Bhatia R ; Gulati S | AFM-TELETHON | 2011Context: Limb girdle muscular dystrophy (LGMD) type 2A is caused by mutation in the gene encoding for calpain-3 resulting into total or partial loss of protein. Diagnosis of LGMD2A, the most prevalent form of LGMD, is obtained by analyzing calpa[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Takada H ; Goto T ; Seikoh K ; Yoshinobu O ; Michio K | AFM-TELETHON | 2011BackgroundInsulin resistance (IR) is a characteristic feature of dysglycaemia in myotonic dystrophy type 1 (DM1). Although the abnormal splicing of insulin receptor mRNA in DM1 muscles reported, the mechanism of IR remained obscure. The aim of t[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Cattin ME ; Ferry A ; Vignaud A ; Decostre V ; Varnous S ; Mougenot N ; Fromes Y ; Bonne G | 2011LMNA gene encodes lamins A/C, ubiquitous proteins of the nuclear envelope. They play crucial role in maintaining nuclear shape and stiffness. If mutated, they lead to muscular and cardiac diseases maybe due, in part, to excessive mechanical stre[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Rafai MA ; Raji L ; Ibnkhribchia R ; Boulaajaj F ; Manaf S ; El Moutawakil B ; Slassi I | AFM-TELETHON | 2011INTRODUCTION : Neurological involevement during sarcoidosis are rare and varied. We report a case of a patient who presented a cranial pachymeningitis and the occurrence of local symptomatic muscular affection that allowed sarcoidosis' diagnosis[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Dorchies O ; Reutenauer-Patte J ; Montet X ; Patthey-Vuadens O ; Ruegg U | 2011DMD is a fatal muscle disorder caused by the absence of dystrophin and characterized by progressive muscle wasting. Weakness of the back muscles often causes postural alterations, impairing patients' respiratory function. Oxidative stress likely[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Le Gall T ; Lindberg M ; Hyde SC ; Gill DR ; Lehn P ; Montier T | 2011Acute unmethylated CG dinucleotide (CpG)-mediated inflammatory response has been shown to be associated with a brief duration of transgene expression in mouse lungs. Indeed, retention of even a single CpG in pDNA was demonstrated to be sufficien[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Rouger K ; Larcher T ; Dubreil L ; Deschamps JY ; Fromes Y ; Le Guiner C ; Jouvion G ; Delorme B ; Lieubeau B ; Ledevin M ; Zuber C ; Leroux I ; Guigand L ; Le Rumeur E ; Cherel Y | AFM-TELETHON | 2011Duchenne Muscular Dystrophy (DMD) is a progressive fatal X-linked recessive disorder of skeletal and cardiac muscles. It represents the most common muscular dystrophy, affecting one in 3,500 male births (Emery, 1991). It is characterized by the [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Laumonier T ; Hoffmeyer P ; Menetrey J | 2011Myoblast transplantation remains a promising therapeutic approach in the treatment of several muscular disorders including Duchenne Muscular Dystrophy. Nevertheless, such therapies are limited by a massive early cell death following injections. [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Stenzel W | AFM-TELETHON | 2011Etiopathogenesis of sarcoidosis, a systemic granulomatous disease, still remains obscure. A multitude of organs affected by systemic sarcoidosis have been described. Skeletal muscles may also be affected, leading to myalgia and weakness. A work-[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Galletta E ; Saletti C ; Pasut A ; Malerba A ; Vitiello L | 2011The interplay between macrophages and muscle precursors is known to be critical for muscle repair and regeneration. In the past, we have already reported that themurine macrophage cell line J774 can produce a macrophage-conditioned medium (MCM) [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Chekiri A ; Abdelhak S ; Hakem D ; Takheroubt K ; Berrah A ; Chaouch M ; Masmoudi AN ; Baba-Ahmed R | 2011INTRODUCTION Idiopathic inflammatory myopathies are a heterogeneous group of diseases including dermatomyositis, polymyositis and inclusion body myositis. It'simportant to diagnose these entities because they are potentially treatable. The aim o[...]Article
Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Martinez Perea MDC | AFM-TELETHON | 2011INTRODUCTION:It has been estimated that more than one person in every 3000 has a serious disabling inherited neuromuscular disorder. There are several scales to measure the stages of motor decline in NMD. Motor Function Measure (MFM) applicable [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Nazzal D ; Truffault F ; Bismuth J ; Berrih-Aknin S | AFM-TELETHON | 2011Regulatory CD4+CD25+ T cells prevent the activation of auto-reactive T cells and play a key role in the induction of peripheral tolerance. We recently showed that regulatory T (Treg) cells are severely defective in the thymus from patients with [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Sadlaoud K | AFM-TELETHON | 2011Mechanisms underlying muscle spasticity: alterations of the neural network and inhibitory synaptic transmission after spinal cord injuryKarina Sadlaoud, Pascale Boulenguez, Patrice Coulon, Laurent Vinay and Hne BrasLaboratoire Plasticitysio-Path[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Borgne FL ; Logerot M ; Guyot S ; Demarquoy J | 2011The metabolic alterations occurring in DMD cells have been little studied. This metabolic aspect of the disease has an obvious interest since metabolic alterations arising in muscle cells lead to an altered energy production that may contribute [...]