Nesse trabalho foi desenvolvida uma plataforma para o condicionamento, digitalização, visualização e gravação de sinais de eletromiografia (EMG). Posteriormente a aquisição, a análise pode ser realizada através de técnicas de processamento de sinais. A plataforma consiste em dois módulos que adquirem sinais de EMG através de eletrodos de superfície, limitam a faixa de frequências de interesse, filtram interferências da rede elétrica e digitalizam os sinais pelo conversor analógico-digital do microcontrolador dos módulos. Deste modo, os dados são enviados para o computador pela interface USB na especificação HID, sendo apresentados em tempo real na forma gráfica e armazenados em arquivo. Como recursos de processamento foram implementadas as operações de módulo do sinal, a determinação do valor eficaz (RMS), a análise de Fourier, filtro digital (IIR) e filtro adaptativo. Foram realizados testes iniciais de avaliação da plataforma com sinais de membros inferiores e superiores para fins de comparação de lateralidade de sinais de EMG. A plataforma aberta destina-se a atividades didáticas e a pesquisa acadêmica, permitindo acrescentar outros métodos de processamento que o pesquisador tenha interesse em avaliar ou outras análises que se façam necessárias. / In this work, a platform to the conditioning, digitizing, visualization and recording of the EMG signals was developed. After the acquisition, the analysis can be done by signal processing techniques. The platform consists of two modules witch acquire electromyography (EMG) signals by surface electrodes, limit the interest frequency band, filter the power grid interference and digitalize the signals by the analogue-to- digital converter of the modules microcontroller. Thereby, the data are sent to the computer by the USB interface by the HID specification, displayed in real-time in graphical form and stored in files. As processing resources was implemented the operations of signal absolute value, the determination of effective value (RMS), Fourier analysis, digital filter (IIR) and the adaptive filter. Platform initial tests were performed with signal of lower and upper limbs with the aim to compare the EMG signal laterality. The open platform is intended to educational activities and academic research, allowing the addition of other processing methods that the researcher want to evaluate or other required analysis.

Les dystrpohies musculaires sont des maladies génétiques rares qui se caractérisent par une dégénérescence musculaire progressive. la Dystrophie Musculaire de Duchenne (DMD) qui est la plus sévère d'entre elles est due à des mutations dans le gène de la dystrophine. Les mécanismes cellulaires impliqués dans le processus de dégénérescence des muscles restent peu compris et aucun traitement efficace n'existe à ce jour. Notre équipe a développé un modèle de la DMD chez le nématode C. elegans qui présente une dégénérescence musculaire progressive. Pendant ma thèse, j'ai caractérisé le processus de dégénérescence musculaire chez ce modèle par microscopie électronique. J'ai également contribué à une étude du rôle des mitochondries dans la dégénérescence musculaire dystrophine-dépendante chez le nématode. Par ailleurs, j'ai étudié l'effet de suppresseurs pharmacologique et génétiques de la dégénérescence musculaire dystrophine-dépendante. Enfin, j'ai pu mettre en évidence que la force exercée par le muscle influence le taux de dégénérescence musculaire. L'ensemble des résultats obtenus au cours de ma thèse, suggèrent que la perte de fonctions de la dystrophine affecte chez le nématode l'intégrité du sarcolemme et des structures d'ancrage des sarcomères et déclenche ainsi une cascade d'événements intracellulaires conduisant in fin à la mort de la cellule musculaire. Ainsi mes travaux dethèse mettent en évidence de nouveau mécanismes cellulaires impliqués dans la dégénérescence musculaire et ouvrent de nouvelles perspectives pour le développement de thérapie visant à cibler les défauts primaires ou secondaires induits par la perte de fonction de la dystrophine / Muscle dystrophies are genetic diseases caraterized by progressive muscle degeneration. Duchenne Muscular Dystrophy (DMD) is the most severe and is due to a mutation in the gene coding the dystrophin protein. The cellular mechanisms implicated in the degenerating process arte not understood yet and there is still no efficient treatment to cure the disease. Our group decvelopped a DMD model in C. elegans that presents progressive muscle degeneration. During my PhD thesis, I characterized the process of muscle degeneration in this model by electron microscopy. I also contribued to an investigation of the role of mitochondira in dystophin-dependant muscle degeneration. I also studied the effect of pharmacological and genetic suppressors of muscle degeneration. Finally, I showed that the force developped by the worm to move influences the level of muscle degeneration. Altogether, the results I obtained during my PhD thesis, suggest that the loss of funciotnof the dystrophin protein affects the integrity of the muscle plasma membrane and the sarcomeres anchoring structures triggering a cascade of intracellular events leading to the muscle cell death in C. elegans. Therefore, my results highlight new cellular mechanisms implicated in the phenomenon of muscle degeneration and open new perspectives for the development of therapies targeting primary and secondary defects induced by the dystrophin loss of function.