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Congrès: Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) (26-30 mai 2008)
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Documents disponibles provenant de ce congrès (565)
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Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Briguet A ; Erb M ; Courdier-Fruh I ; Barzaghi P ; Santos G ; Herzner H ; Lescop C ; Siendt H ; Henneboehle M ; Weyermann P ; Magyar J ; Dubach-Powell J ; Metz G ; Meier T | 2008Dystrophin deficiency is the underlying molecular cause of progressive muscle weakness observed in Duchenne muscular dystrophy (DMD). Loss of functional dystrophin leads to elevated levels of intracellular Ca2+, a key step in the cellular pathol[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Tonson A ; Ratel S ; Le Fur Y ; Cozzone P ; Bendahan D | 2008The purpose of this investigation was to determine whether maturation affects the relationship between muscle size and maximal strength and to investigate the reasons accounting for the discrepancies among previous studies. Methods: Maximal isom[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Rauscent H ; Bérard C ; Humbertclaude V ; Gautheron V ; Richelme C | 2008Concerning multidisciplinary care of Duchenne Muscular Dystophy (DMD), the prevention of scoliosis remains a priority. Spinal surgery is considered as the treatment of choice and early instrumentation and fusion are widely proposed. The objectiv[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Noirez P ; Mouly V ; Fiszman M ; Keller A ; Dubois C ; Alameddine HS | 2008Fibrosis, characterized by excessive accumulation of extracellular matrix (ECM), is a hallmark of muscle biopsies in several muscular dystrophies such as Congenital (CMD) or Duchenne Muscular Dystrophies (DMD). Cell therapy trials have shown tha[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Orlikowski D ; Laforet P ; Pellegrini N ; Prigent H ; Monnet A ; Carlier P ; Carlier R ; Eymard B ; Lofaso F ; Annane D | 2008Efficacy of Enzyme Replacement (ERT)Therapy is not fully assessed in severe late-onset forms of Pompe disease (SLO Pd). The aim of the study is to assess efficacy and tolerance of ERT in SLO Pd in a Prospective, open-label, single arm, monocente[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Turin E ; Hoff H ; Gatti F ; Winders T ; Singh R ; Starke J ; Rutter J ; Bledsoe C ; Lavin J ; Palmieri B ; Carlson CG | 2008Ursodeoxycholic acid (UDCA) is in current clinical use for the treatment of biliary cirrhosis and has been shown to reduce nuclear p65 activation in HeLa cells expressing elevated glucocorticoid receptor (Miura et al., J. Biol. Chem., 276(50), 4[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Bernardi H ; Gay S ; Fedon Y ; Bolzec T ; Bacou F | 2008The molecular signals that regulate satellite cell function remain largely obscure. However, it was recently demonstrated that Wnts participate in the temporal control of satellite cell expansion versus differentiation during adult muscle regene[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Lostal W ; Bourg N ; Roudaut C ; Miyake K ; Mcneil P ; Bartoli M ; Richard I | 2008Dysferlinopathies are recessive muscular disorders caused by defects in dysferlin. Genetic mutations are responsible for two major phenotypes: Limb Girdle Muscular Dystrophy type 2B and Distal Miyoshi Myopathy. These skeletal muscle diseases are[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Steffensen B ; Mayhew A ; Aloysius A ; Eagle M ; Mercuri E ; Messina S ; Mazzone E ; Nadeau A ; Main M ; Scott E ; Werlauff U ; Werge B ; Glanzmann A ; Muntoni F | 2008The EK scale was developed as a clinical tool to assess functional ability and to determine the need for and impact of intervention in the non-ambulatory stages of Duchenne muscular dystrophy (DMD) and in spinal muscular atrophy (SMA). EK is a c[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Blais F ; Léjard V ; Maro GS ; Gilardi-Hebenstreit P ; Rossert J ; Duprez D | 2008The molecular mechanisms underlying tendon formation during vertebrate embryogenesis are still largely unknown. In Drosophila, tendon differentiation relies upon the transcription factor stripe, an Early growth response (Egr)-like transcription [...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Giroux-Metgès MA ; Hogrel JY ; Fiszman MY ; Fromes Y | 2008CHF147 hamster strain is an animal model of delta-sarcoglycan deficiency. Skeletal muscular dystrophy features observed in this model are very similar to those described in humans with more severe disease in proximal than distal muscles. Muscle [...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Boerio D ; Lefaucheur JP ; Bassez G ; Hogrel JY | 2008Fatigue frequently occurs in various neuromuscular disorders specially in myotonic dystrophy type 1 (DM1). However its pathophysiology remains unclear. This study aimed to investigate the effects of exercise-related fatigability on central and p[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Jaconi M | 2008Cell transplantation therapy is an attractive strategy in the treatment of myocardial infarction. Given the present lack of a good cardiogenic source for transplantation among adult stem cells, lots of studies are presently focusing on methodolo[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Desguerre I ; Barbet P ; Gherardi R ; Christov C | 2008The primary muscle fiber injury in DMD is due to total absence of dystrophin. With evolution of the disease, an increasing extent of myofibrosis, the precise mechanisms of which remain to be fully determined, likely accelerates myofiber death. I[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Malerba A ; Vitiello L ; Segat D ; Dazzo E ; Frigo M ; Scambi I ; Boldrin L ; Martelli L ; Pasut A ; Romualdi C ; Baroni D | 2008Skeletal muscle regeneration relies onto a specific population of myogenic precursors, named satellite cells. Inflammation also has a determinant role, as upon injuring macrophages are attracted by the damaged myofibers and the activated satelli[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Nivet AL ; Plancheron A ; Lustremant C ; Peschanski M ; Monville C | 2008Duchenne muscular dystrophy (DMD) is a genetic disease with an X-linked recessive pattern of inheritance. It affects one in 3,500 boys at birth. Muscular dystrophies are caused by mutation of the same gene encoding for the dystrophin. This prote[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Mongue-Din H ; Liu JM ; Salmon A ; Fiszman MY ; Wdzieczak-Bakala J ; Fromes Y | 2008The chronically failing heart is characterized by alterations in tissue structure, particularly fibrous tissue formation, responsible for the loss of myocardial compliance. Furthermore, rhythm disturbances are commonly observed. The underlying a[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Doglio L ; Pernigotti I ; Tacchino C ; Pedemonte M ; Scapolan S ; Minetti C | 2008DMD patients walk with a non-physiological pattern showing a initially weakness of antigravitary muscles, followed by loss of walking capability. Initially, DMD patients do not show clinical signs of pathological pattern, that become manifest wi[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Jenny C ; Albert V ; Vovard F ; Denèfle P ; Merten OW | 2008Myogenic progenitor cells are promising tools for cell therapy to treat neuromuscular disorders. But a challenging question is their amplification to a satisfying scale for clinical applications. Until now, most of myogenic progenitor cells have[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Vattemi G ; Marini M ; Mechref Y ; Meneguzzi A ; Tonin P ; Grigoli L ; Di Chio M ; Tedesco V ; Lovato L ; Filosto M ; Scarpelli M ; Chiamulera C ; Minuz P ; Novotny M ; Tomelleri G | 2008Mitochondrial diseases (MD) are multisystemic disorders, mostly affecting central nervous system, skeletal and cardiac muscle. To evaluate the hypothesis that in MD the vessel wall, in particular the vascular endothelium, may be affected by incr[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Belicchi M ; Meregalli M ; Razini P ; Cattaneo A ; Farini A ; Iacchetti E ; Porretti L ; Milani P ; Bresolin N ; Torrente Y | 2008The use of stem cells in regenerative medicine and cell-based therapies offers immense potential in diseases witch have currently no treatment such as Duchenne muscular dystrophy. A limitation to the use of CD133+ for a therapeutic application i[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Falorni M ; Volpi L ; Calsolaro V ; Bonanni E ; Monzani F ; Emdin M ; Siciliano G | 2008Excessive daytime sleepiness (EDS) is common in patients with Myotonic Dystrophy type 1(DM 1) and is a major factor that interferes with the social life of the disease. There are contrasting opinions in literature about the mechanism of EDS in D[...]Article
Exercise-induced rhabdomyolysis as isolated clinical expression of COQ10 deficiency. A new phenotype
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; González-Pérez P ; Rivas-Infante E ; Sánchez-Alcázar JA ; Navas Lloret P ; Bautista-Lorite J | 2008OBJECTIVES: We present a new phenotype associated to CoQ10 deficiency characterised by a late-onset recurrent myoglobinuria and exercise intolerance without muscle weakness nor CNS involvement. CoQ10 supplementation was introduced and by now the[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Navarro C ; Farini A ; Meregalli M ; Belicchi M ; Parolini D ; Razini P ; Krahn M ; Wein N ; Bourg N ; Bartoli M ; Richard I ; Torrente Y | 2008Mutations in gene encoding Dysferlin are involved in Limb-gird Muscular Dystrophy type 2B (LGMD-2B) and and Miyoshi myopathy (MM), both diseases are characterized by progressive weakness and wasting of skeletal muscles. Dysferlin is abundantly e[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Rahbek J | 2008Most clinicians, researchers, health care advocates, policy makers, and even third-party payers agree - on a conceptual level - that the measurement of QoL is an important consideration in evaluating the effectiveness of medical rehabilitation i[...]