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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 (478)
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Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Csibi A ; Lagirand-Cantaloube J ; Offner N ; Leibovitch MP ; Barboiron C ; Picard B ; Leibovitch S | 2008Skeletal muscle size depends upon a dynamic balance between anabolic and catabolic processes. The E3 ubiquitin-ligase MAFBx/Atrogin-1 is upregulated during muscle atrophy caused by a variety of conditions, including cancer, AIDS, stress, diabete[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Riederer I ; Negroni E ; Bencze M ; Wolff A ; Savino W ; Butler-Browne GS ; Silva-Barbosa S ; Mouly V | 2008Myoblast transplant therapy (MTT) can be envisioned as a clinical alternative in the treatment of certain diseases. The problems that still remain to be solved to improve the efficiency of MTT are the massive early cell death, the limited prolif[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Benabdallah BF ; Bouchentouf M ; Rousseau J ; Tremblay JP | 2008Duchenne muscular dystrophy is caused by the absence of functional dystrophin protein, leading to the myofiber membrane instability and progressive muscle atrophy. Myoblast transplantation in dystrophic muscles is a potential therapy for the dis[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Gross DA ; Vignaud L ; Da Rocha Rodrigues S ; Gjata B ; Charles S ; Georger C ; Scherman D ; Israeli D | 2008Transplantation of muscle precursor cells (MPC) into dystrophic muscle is a major therapeutic approach in muscular dystrophies. However the clinical benefit of this approach is seriously compromised by the low survival rate of the transplanted c[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Ramachandran U ; Saleki K ; Marlow G ; Anderson L ; Bashir R | 2008The dysferlin gene is mutated in autosomal recessive Miyoshi myopathy, Limb Girdle Muscular Dystrophy type 2B (LGMD2B) and distal anterior compartment myopathy resulting in deficiency of the protein in patient muscle. Dysferlin is a sarcolemmal [...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Mercuri E | 2008Increasing attention has been devoted in the last few years to the use of functional scales and other assessments of muscle function and strength in neuromuscular disorders. A range of good functional outcome measures already exist which are rea[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Frédéric M ; Clot F ; Blanchard A ; Dhaenens CM ; Lesca G ; Cif L ; Durr A ; Vidailhet M ; Sablonnière B ; Calender A ; Brice A ; Claustres M ; Tuffery-Giraud S ; Collod-Béroud G | 2008Early-Onset Torsion Dystonia (EOTD) are rare movement disorders developing in childhood with a neurological origin. They begin in a limb and potentially spread to other parts of the body to become generalized. Molecular defect is known for only [...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; van Zoggel J ; Alabanese P ; Jacobs MS ; Courty J ; Papy-Garcia D ; Morin C ; Martelly I | 2008Overproduction of reactive oxygen species has been implicated in cellular defect that may lead to apoptosis. Skeletal muscle cells are frequently submitted to oxidative stress upon excessive exercise or in disease. Although cells withstand oxida[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Gasnier E ; Bigot A ; Mouly V ; Furling D | 2008Myotonic Dystrophy type I (DM1) is caused by a CTG expansion in the 3’-UTR of the DMPK gene and is characterized by progressive muscle weakness and wasting. Large CTG repeats affect the differentiation program and we have showed that the prolife[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Klein AF ; Ebihara M ; Alexander C ; Dicaire MJ ; Rouleau GA ; Brais B | 2008Oculopharyngeal muscular dystrophy (OPMD) is a late-onset autosomal dominant disease caused by expansions of a (GCN)10/polyalanine tract in the gene coding for the Poly(A) Binding Protein Nuclear 1 (PABPN1). The pathological hallmark of the dise[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Krahn M ; Wein N ; Lostal W ; Bourg-Alibert N ; Nguyen K ; Courrier S ; Vial C ; Labelle V ; De Petris D ; Borges A ; Mattéi MG ; Roudaut C ; Miyake K ; Mcneil P ; Cau P ; Leturcq F ; Bartoli M ; Richard I | 2008Primary dysferlinopathies are a heterogeneous group of autosomal recessive muscular dystrophies, caused by mutations in the large-sized DYSF gene encoding dysferlin. Dysfunction of dysferlin causes deficient sarcolemmal repair and leads to muscl[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Poitelon Y ; Baudot C ; Hamadouche T ; Delague V | 2008Lamins, a class of intermediate filaments, are major components of the nuclear lamina, a filamentous network underlying the inner face of the nuclear membrane. A-type Lamins are encoded by the same gene: LMNA, regulated by the AP1 complex (c-Fos[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Parolini D ; Meregalli M ; Belicchi M ; Farini A ; Razini P ; Maciotta S ; Torrente Y | 2008Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease due to a deficiency in dystrophin, a 427kDa protein located at the sarcolemma and acting as a linker between cytoskeleton and extracellular matrix Several observations sugg[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Sifi Y ; Sifi K ; Abadi N ; Benlatreche C ; Koeing M ; Hamri A | 2008INTRODUCTION Myotonic dystrophy (MD1).is the most common form of adult muscular dystrophy with autosomal dominant transmission. It is an inherited disease in which there is an abnormal expansion of CTG trinucleotide repeat at 19q13.3. It is mani[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Sifi Y ; Sifi K ; Boulefkhad A ; Abadi N ; Benlatreche C ; Hamri A | 2008Introduction: Pompe disease (PD), is caused by a deficiency of the enzyme lysosomal, acid-glucosidase (GAA) resulting in the accumulation of glycogen primarily in muscle tissue. The clinical presentation of (PD) is variable with respect to the a[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Desguerre I ; Christov C ; Mayer M ; Zeller R ; Bécane HM ; Leturcq F ; Chelly J ; Gherardi R | 2008Innovative therapies are presently being developed for DMD. Evaluating their effect will require precise knowledge of both the natural history and factors influencing the course of a disease which can be no longer considered as homogeneous in te[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Nouioua S ; Magy L ; Hamadouche T ; Rautenstrauss B ; Grid D ; Assami S ; Vallat JM ; Tazir M | 2008Giant axonal neuropathy (GAN) is a recessively inherited neurological disorder affecting both central and peripheral nervous system. The main pathological hallmark of the condition is abnormal accumulation of intermediate filaments in giant axon[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Birouk N ; Bouhouche A ; Belaidi H ; Benomar A ; Azzeddine H ; Dubourg O ; Maisonobe T ; Yahyaoui M ; Le Guern E ; Ouazzani R | 2008Mutations in GDAP1 gene located in 8q13 chromosome have been identified in families with either axonal or demyelinating form of autosomal recessive Charcot-Marie-Tooth (CMT) disease. Twenty five patients belonging to 14 Moroccan consanguineous f[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Birouk N ; Bouhouche A ; Belaidi H ; Slassi I ; Benomar A ; Azzeddine H ; Maisonobe T ; Dubourg O ; Yahyaoui M ; Le Guern E ; Ouazzani R | 2008The most frequent form of axonal autosomal recessive Charcot-Marie-Tooth neuropathy is a laminopathy. R289C is the only mutation descried so far in all reported families. This is a phenotype study of patients with R289C mutation in Lamin A/C gen[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Pietri Rouxel F ; Gentil C ; Ferry A ; Vignaud A ; Garcia L | 2008Adipose infiltration is a characteristic common to many muscular dystrophies. In certain cases, the infiltration is such that all of the muscle fibers are replaced by adipocytes, preventing or at least limiting, the rational of gene or cell ther[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Goudeau B ; Simon S ; Casteras-Simon M | 2008Myofibrillar myopathies (MFM) are rare inherited neuromuscular disorders characterized by adult onset and the accumulation of aggregates containing desmin in cardiac and/or skeletal muscle cells. MFM patients present clinical and genetic heterog[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Thorel D ; Méchin D ; Beroud C ; Rivier F ; Coubes C ; Jouk PS ; Leturcq F ; Cossee M ; Tuffery-Giraud S ; Claustres M ; Khau Van Kien P | 2008In the families with a reported case of dystrophinopathy, pedigree analysis with measurement of blood creatine phosphokinase (CK) are commonly used for genetic risk assessment of relatives. Several affected cases in the same family across genera[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Llorens A ; Diaz-Ramos MA ; Lopez-Alemany R | 2008The Plasminogen Activation (PA) system is a group of serin-proteases that plays an important role in a wide range of biological processes in which tissue remodelling takes place. Plasmin, generated by activation of its zymogen plasminogen, is a [...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Diaz-Ramos A ; Llorens A ; Lopez-Alemany R | 2008The plasminogen activation (PA) system is a group of serine proteases that participate in tissue remodeling by degrading most of components of the extracellular matrix. Different studies have shown that PA system components (plasminogen and urok[...]Article
Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ; Leblanc A ; Bondoin L ; Marcorelles P ; Blanchard C ; Zagnoli F | 2008In polymyositis, CD8 cells recognise unknown antigen linked to HLA. But in some cases, this antigen can be identified. A sixty years old Caribbean woman complained for progressive walking disability for ten years, without pain. In 2007, physical[...]