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IXèmes Journées Annuelles de la Société française de Myologie (SFM) (3-5 novembre 2011; Angers) ; Gagnon C ; Mathieu J | 2011La prise en charge des personnes atteintes de dystrophie myotonique de type 1 (DM1) est un modèle de complexité à plusieurs niveaux incluant la prise en charge des atteintes multisystémiques, la personnalité atypique, l'environnement social dist[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Cacheux M ; Fauré J ; Brocard J ; Monnier N ; Lunardi J ; Marty I | 2011Muscle contraction is achieved when an efficient excitation signal at the plasma membrane triggers intracellular calcium release. This process called "excitationcontraction (E-C) coupling" relies on a multimolecular protein complex, the calcium [...]Article
Leturcq F ; Cossee M ; Deburgrave N ; Orhant L ; Kaplan JC ; Stojkovic T ; Laforet P ; Behin A ; Brais B ; Chelly J | 2011Mutations in the anoctamin 5 (ANO5) gene , which encodes a putative calcium-activated chloride channel, have been described in patients with limb-girdle muscular dystrophy (LGMD2L) and distal myopathies. The mutations described so far are point [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Lepper C ; Partridge T ; Chen-Ming F | AFM-TELETHON | 2011Skeletal muscle tissue is prone to damage from acute physical trauma such as sport injuries as well as from daily wear and tear: this is managed by its tremendous capacity to repair itself. Cell transplantation and lineage tracing studies have d[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Kostallari E ; Lafuste P ; Baba-Amer Y ; Gherardi RK | 2011Sublaminar location has been the cornerstone of both definition and identification of muscle satellite cells (mSCs), but little attention has been paid to the anatomic organization of the mSC niche, and interplays of mSCs with their neighborhood[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Sarrazin N ; Bangratz M ; Devaux J ; Zambroni D ; Echaniz-Laguna JA ; Rene F ; Boerio D ; Davoine CS ; Fontaine B ; Feltri ML ; Benoit E ; Nicole S | 2011Schwartz-Jampel syndrome (SJS) is a recessive disorder characterized by spontaneous activity in the rest EMG that may result from peripheral nerve hyperexcitability (PNH). SJS results from a lack of perlecan, the major proteoglycan of basement m[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Daussin F ; Godin R ; Ascah A ; Deschênes S ; Petrof B ; Burelle Y | 2011We examined the mitochondrial phenotype in skeletal muscle in the early phase of Duchenne muscular dystrophy (DMD), and determined whether upregulation of mitochondrial biogenesis via PGC-1_ transfection is beneficial in the mdx mice, a murine m[...]Article
Hentati F ; Rigolet A ; Behin A ; Romero NB ; France L ; Pascal L ; Maisonobe T ; Amouri R ; Haddad H ; M.Audit ; Montus M ; Masurier C ; Gjata B ; Georger C ; Cherai M ; Carlier P ; Hogrel JY ; Herson A ; Lemoine FM ; Klatzmann D ; Sweeney L ; Mulligan RC ; Eymard B ; Caizergues D ; Voit T ; Herson S | AFM-TELETHON | 2011BACKGROUND Gamma-sarcoglycanopathy or limb girdle muscular dystrophy type 2C (LGMD 2C) is an untreatable disease caused by autosomal recessively inherited mutations of the -sarcoglycan gene (SGC). METHODS Nine non-ambulatory LGMD2C patients (2 M[...]Article
AFM-TELETHON 2011Mesoangioblasts are recently characterized progenitor cells, associated with the vasculature and able to differentiate into different types of solid mesoderm, including skeletal muscle (Minasi et al. Development 129, 2773, 2002). When wild type [...]Article
IXèmes Journées Annuelles de la Société française de Myologie (SFM) (3-5 novembre 2011; Angers) ; Bassez G | 2011Près d'un siècle après la description fondatrice d'Hans Steinert, la génétique moléculaire a provoqué l'émergence d'une nouvelle entité, la dystrophie myotonique de type 2 (DM2) au sein d'une nomenclature revisitée. Elle est comme la DM1 causée [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Briggs D ; Morgan J ; Boldrin L | 2011Satellite cells are the principal muscle stem cell, however not all satellite cells contribute equally to muscle regeneration. It is thought that there is a sub-population of satellite cells which is more 'stem cell like' than others. We aim to [...]Article
Generation of skeletal muscles with forms adapted to their function is essential for normal movement, and involves developmental mechanisms such as those regulating muscle diversification and migration. Understanding these developmental processe[...]Article
AFM-TELETHON 2011The lack of existing models of pathologic tissues has rendered many important questions in disease pathogenesis inaccessible. Human embryonic stem cells derived from affected embryos during a pre-implantation diagnostic (PGD), as well as the tec[...]Article
Alao Maroufou J ; Leturcq F ; Sagbo G ; Urtizberea JA ; Réseau Ouest Africain de prise en charge des Myopathies | 2011INTRODUCTION : Les maladies neuromusculaires sont présentes partout dans le monde. Peu de cas ont été rapportés en Afrique subsaharienne. Des professionnels africains réunis au sein du Réseau Ouest Africain de prise en charge des Myopathies (ROA[...]Article
Valadares M ; Zucconi E ; Secco M ; Bueno Junior C ; Brandalise V ; Assoni A ; Gomes J ; Landini V ; Andrade T ; Lima B ; Vainzof M ; Zatz M | AFM-TELETHON | 2011The possibility to use stem-cells in the future as a therapy for progressive muscular dystrophies has been of great interest. However, many questions still need to be addressed. What is the best way of delivery: local or systemic? What are the b[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Cassinelli LM ; Maciotta S ; Gandolfi F ; Forcato M ; Bicciato S ; Meregalli M ; Torrente Y | 2011Duchenne muscular dystrophy (DMD) is a common X-linked disease characterized by frameshift mutations in the dystrophin gene. Among the molecular mechanisms potentially involved in DMD, we focused our attention on microRNAs (miRNAs) a new class o[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Pinto Mariz F ; Barthelemy I ; Yada E ; Voit T ; Silva-Barbosa SD ; Savino W ; Butler Browne G ; Blot S | 2011It is well known that the disease course in Duchenne muscular dystrophy (DMD) patients is heterogeneous, varying from patient to patient. Such heterogeneity is also seen in the Golden Retriever Muscular Dystrophy (GRMD) dogs. This is a drawback [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Diguet N ; Mallat Y ; Ladouce R ; Clodic G ; Tritsch E ; Blanc J ; Larcher JC ; Delcayre C ; Samuel JL ; Friguet B ; Bolbach G ; Li Z ; Mericskay M | 2011RATIONALE: Alterations in the balance between sarcomeric and extra-sarcomeric cytoskeleton as well as energetic proteins are involved in the remodelling of cardiomyocytes cytoarchitecture in dilated cardiomyopathy (DCM). OBJECTIVE: Inactivation [...]Article
AFM-TELETHON 2011During development, founder stem cells proliferate, and cell cycle exit is generally restricted to differentiating cells. In contrast, adult stem cells, can assume different cellular states, either quiescence or proliferating. After embryonic de[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Benedetti S ; Magagnotti C ; Bachi A ; Zerbini G ; Fattore E ; Riba M ; Previtali S ; M.Ferrari ; Andolfo A | 2011Lamins A/C, encoded by the LMNA gene, are intermediate filaments of the nuclear lamina playing multiple roles in nuclear integrity, chromatin organization and transcriptional control. Defects in the LMNA gene have been associated with a growing [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Denard J ; Beley C ; Kotin R ; Samulski J ; Moullier P ; Voit T ; Garcia L ; Svinartchouk F | 2011Despite the well-established safety and efficacy of rAAV vectors for in vivo gene transfer, there is still little information concerning the fate of vectors after systemic delivery. By using a proteomic approach, we screened for serum proteins i[...]Article
Hammer C ; Puymirat J ; Bassez G ; Allain F ; Furling D ; Charlet Berguerand N | AFM-TELETHON | 2011Myotonic Dystrophy (DM) is the most frequent muscular dystrophy in adult. Myotonic Dystrophy type 1 (DM1) is caused by an expansion of CTG repeats located within the 3'-untranslated region of the DMPK gene, while DM2 is caused by an expansion of[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Barthelemy I ; Thibaud JL ; Aguilar P ; Le Chevoir M ; Blot S | 2011Most of DMD (Duchenne muscular dystrophy) patients die from respiratory failure. Their canine homologues, GRMD (Golden retriever muscular dystrophy) dogs, also develop respiratory dysfunction. The evaluation of this function seems essential duri[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Carlier PG ; Loureiro De Sousa P ; Azzabou N ; Wary C ; Carlier RY | 2011Muscle imaging, and in particular NMR imaging, will play an increasingly important role, together with dynamometry and actimetry, for the evaluation of therapeutic interventions in patients with neuro-muscular disorders. To fulfil this mission, [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Borel P ; Guerchet N ; Tanniou G ; Bloch R ; Roche J ; Richard I ; Stockholm D | 2011Sarcolemmal disruptions in myofibers can represent a physiologic response to a particular mechanic stress like lengthening contractions. These membrane tears result in intracellular changes that play a crucial role in physiopathology of the dise[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Decorte N ; Cabrol L ; Drouet M ; Azzabou N ; Carlier PG | 2011Purpose. Abnormal skeletal muscle enhancement post Gd-contrast agent (Gd-CA) injection is a common feature on NMR images in inflammatory diseases but also inchronic interstitial fibrosis or in conditions where sarcolemma permeability is increase[...]Article
Fardeau M, Author | 2011Un grand nombre de maladies neuromusculaires d'origine génétique touchent les muscles du visage, et donc les modalités d'expression faciale des personnes atteintes. Les mécanismes, les variantes topographiques et sémiologiques de cette atteinte [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Hardeman E ; Lee A ; Joya J ; Gunning P | 2011We are developing a strategy for the selective engraftment of stem cells in a solid tissue. It is based on the forced expression in transplanted cells of a mutant form of methylguanine methyltransferase [MGMT(P140K)] which can confer resistance [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Castel D ; Sambasivan R ; Tajbakhsh S | 2011Satellite cells play a major role in postnatal growth and regeneration of skeletal muscle. Thus, satellite cells exhibit several attributes of stem cells: quiescence, selfrenewal and regenerative potential. Since all skeletal muscle satellite ce[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Lacourpaille L ; Bouillard K ; Hug F ; Hogrel JY ; Nordez A | 2011Non-invasive measurements are required for monitoring neuromuscular disorders (NDM) and following the effects of rehabilitation/therapy programs. Since it provides localized elastic modulus (i.e. stiffness) measurements, elastographic methods co[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Domi T ; Porrello E ; Velardo D ; Capotondo A ; Triolo D ; Ruegg MA ; Biffi A ; Comi G ; Quattrini A ; Previtali S | 2011CMD is characterized by progressive wasting muscular dystrophy and dysmyelinating neuropathy with variable involvement of the central nervous system, which may lead to severe disability in early childhood. Among this group, laminin 2deficient CM[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Buyse G ; Goemans N ; van den Hauwe M ; Thijs D ; Wei H ; Rummey C ; Meier T ; Mertens L | 2011Background: A 12-month double-blind, randomized, placebo-controlled phase II study in 21 patients with DMD (DELPHI study) has indicated that idebenone (Catena®, 450 mg/d) may improve cardiac and respiratory function in 8-16 year old patients wit[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Ben Yaou R ; Beugnet C ; Chikhaoui K ; Hamroun D ; Deburgrave N ; Kaplan JC ; Beroud C ; Bonne G ; Leturcq F | 2011In 1966, A. Emery and F. Dreifuss described a large family from Virginia, where affected males showed an unusual type of X-linked condition characterised by muscular dystrophy with early joint contractures and cardiac disease, thus starting the [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Deltour L ; Tajbakhsh S ; Poirier F | 2011Galectins belong to a family of soluble proteins that can be found both extracellularly and intracellularly. In vivo, their localisation is highly regulated depending on physiological situations. Several studies has established that, in addition[...]Article
Cacchiareli D ; Cazzella V ; Cesana M ; Incitti T ; Pinnaro C ; Legnini I ; Morlando M ; Bozzoni I | AFM-TELETHON | 2011MicroRNAs are recognized as important regulators of gene expression in the differentiation commitment of several cell types and have been shown to occupy very high hierarchical positions in the cascade of regulatory events controlling cell speci[...]Article
Hubert L ; Romero NB ; Viollet L ; Laforet P ; Mathew S ; Bekri S ; Kirk E ; Peters H ; McGill JJ ; Glamuzina E ; von der Hagen M ; Alexander IE ; Kirmse B ; Vaz FM ; Munnich A ; Elpeleg O ; Delahodde A ; de Keyzer Y ; de Lonlay P | AFM-TELETHON | 2011Introduction: Main metabolic causes of myopathies are fatty acid oxidation (FAO) deficiencies. LPIN1 defects have been involved in severe and early rhabdomyolysis. Lipin1 plays a dual role, as a phosphatidate phosphatase 1 (PAP1) and as a transc[...]Article
The plasminogen activation (PA) system is a mechanism extensively used by the cell for the generation of proteolytic activity in the extracellular matrix, where it contributes to tissue remodeling in a wide range of physiopathological processes.[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Christodoulou-Vafeiadou E ; Favier M ; Navière A ; Maire P ; Guillet-Deniau I | 2011Intramyocellular lipid deposition contributes to muscle insulin resistance, particularly in obese, type 2 diabetic patients, or in patients with myotonic dystrophy. On the other hand, disruption of Wnt signaling caused transdifferentiation of my[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Boutonnet C ; Bello NF ; Lamsoul I ; Métais A ; Mouly V ; Lutz P ; Moog-Lutz C | 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)) ; Castets P ; Bertrand A ; Beuvin M ; Ferry A ; Grand FL ; Castets M ; Chazot G ; Rederstorff M ; Krol A ; Lescure A ; Romero NB ; Guicheney P ; Allamand V | 2011In humans, selenoprotein N (SelN) deficiency, due to mutations in the SEPN1 gene, causes a group of inherited neuromuscular disorders termed SEPN1-Related Myopathies. These congenital diseases are characterized by an early onset generalized musc[...]Article
AFM-TELETHON 2011Myotonic Dystrophy type 1 (DM1) also called Steinert disease is one of the most common form of inherited neuromuscular disorders in adults characterized by progressive muscle weakness and wasting, myotonia as well as many other multisystemicdefe[...]Article
Bohm J ; Gras S ; Müller J ; Plewniak F ; Keime C ; Vicaire S ; Jost B ; Mandel JL ; Biancalana V ; Laporte J | AFM-TELETHON | 2011Neuromuscular diseases (NMD) are debilitating diseases with a strong impact on the individuals and the society. Despite tremendous research and clinical efforts, the molecular causes of NMD are still unknown for about 40% of patients, and additi[...]Article
AFM-TELETHON 2011Maintenance of muscle mass and physiology is essential for general health. Disuse (e.g. immobilization, denervation, and microgravity), inherited neuromuscular disorders, and aging all result in debilitating loss of skeletal muscle. Given the si[...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Picchio L ; Taghli O ; DaPonte JP ; Jagla C | 2011Myotonic dystrophy type 1 (DM1) is a dominant neuromuscular disorder affecting 1/8000 individuals worldwide. This disease is caused by an unstable expansion of CTG trinucleotide repeats located in the 3' untranslated region of the dystrophy myot[...]Article
Kabzinska D ; Kabzinska D ; Niemann A ; Drac H ; Huber N ; Suter U ; Hausmanowa-Petrusewicz I | AFM-TELETHON | 2011Nonsense-truncating mutations acting by a loss of function mechanism, located in the GDAP1 gene, have been thought to be associated with severe forms of AR-CMT2 disease.The patients harboring nonsense GDAP1 gene mutations usually manifest with f[...]Article
AFM-TELETHON 2011Distal myopathy with rimmed vacuoles (DMRV), also called hereditary inclusion body myopathy (hIBM), is an autosomal recessive debilitating disorder affecting young adults with the age of onset ranging from 15 years to late thirties. The disease [...]Article
AFM-TELETHON 2011Skeletal muscle atrophy is a significant co-morbidity seen in a variety of diseases, including Congestive Heart Failure, renal failure, cancer, and AIDS. Even during simple muscle inactivity, such as when a cast is put on a limb, the affected mu[...]Article
Six1 and Six4 are homeoproteins of the Six/sine oculis family. These proteins are expressed continuously during muscle development: in the dermomyotomal hypaxial myogenic progenitors, they control Pax3 expression and myogenic progenitors fate; i[...]Article
Petiot J ; de Dreuzy E ; Richard I ; Stockholm D | 2011Dysferlin deficient muscle fibers have been shown to be defective in membrane repair after a laser wounding damage of the sarcolemme. In vivo, sarcolemmal membrane disruption can either lead to fiber necrosis or repair but little is known about [...]Article
4th International Congress of Myology, 4ème colloque international de Myologie (9-13 mai 2011; Lille (France)) ; Coletti D ; Aulino P ; Mericskay M ; Li Z ; Adamo S | 2011In humans, selenoprotein N (SelN) deficiency, due to mutations in the SEPN1 gene, causes a group of inherited neuromuscular disorders termed SEPN1-RelatedMyopathies. These congenital diseases are characterized by an early onset generalized muscl[...]