La nature intermittente et aléatoire des sources renouvelables, telles que le photovoltaïque et l’éolien, nécessite un complément de stockage, tel une batterie et un système de secours énergétique, tel un générateur diesel, en particulier dans un système autonome. En ce qui concerne le générateur diesel, il a besoin d'un certain temps pour démarrer et il ne peut pas donner immédiatement la puissance nécessaire, en raison de son comportement dynamique. Alors, la qualité de l'énergie est abaissée pendant cette période en raison du manque de puissance. Par conséquent, pendant la période de démarrage du générateur diesel, un supercondensateur est suggéré pour équilibrer la puissance en raison de sa réponse rapide et de sa densité de puissance élevée. Une stratégie de contrôle de puissance est proposée pour réaliser la coordination entre le générateur diesel et le supercondensateur. La simulation et les résultats expérimentaux montrent que la stratégie de contrôle proposée est capable de réguler la tension du bus continu dans des limites acceptables et d’alimenter la charge pendant la sous production d'énergie renouvelable ou lors d'augmentation de la demande de la charge. De plus, le supercondensateur peut également être utilisé pour surmonter les limites de stockage électrochimique telles que son état de charge et son courant maximal. Ainsi, cette thèse propose le contrôle de puissance en temps réel pour un micro réseau continu avec un système hybride photovoltaïque-batterie-supercondensateur-diesel, visant à répondre à la demande de puissance de charge avec fiabilité et à stabiliser de la tension du bus continu. La simulation et les résultats expérimentaux montrent également que la stratégie de contrôle améliore les performances dynamiques et statiques du micro réseau continu pour différentes conditions de fonctionnement. De plus, afin de minimiser le coût énergétique du groupe diesel, le coût du carburant et la consommation de carburant sont analysés à travers plusieurs tests expérimentaux. Par conséquent, la valeur optimale de sa production d'énergie est déduite et appliquée dans une nouvelle stratégie de gestion de la puissance est proposée. Cette stratégie peut atteindre l'objectif de maximiser l'utilisation de l'énergie photovoltaïque et de prendre en compte la caractéristique de démarrage lent et le coût énergétique du générateur diesel. Les simulations et expérimentations sont réalisées en utilisant des données photovoltaïques réelles pour illustrer les performances et le comportement du système hybride. Les résultats obtenus vérifient l'efficacité de cette stratégie. De plus la comparaison avec la stratégie de gestion de la puissance précédente, dans laquelle le coût d’énergie du générateur diesel n'est pas pris en compte, démontre que la nouvelle stratégie de gestion peut réduire le coût total du système de puissance à courant continu hybride. / The intermittent and random nature of renewable sources, such as photovoltaic and wind turbine, asks for the complement of storage, such as battery and back-up energy, such as diesel generator, especially in a standalone power system. Concerning the diesel generator, it needs some time to start up and cannot immediately offer the needed power, due to its dynamic behavior. Hence, the power quality is lowered down during this period because of the shortage of power. Therefore, during the period of the diesel generator starting up, a supercapacitor is suggested to compensate the power balance because of its fast response and high power density. A power control strategy is proposed to achieve the coordination between diesel generator and supercapacitor. Both simulation and experimental results show that the proposed control strategy is able to regulate the DC bus voltage within the acceptable limits and supplying the load during the renewable power under generation or load step-increase situations. In addition, the supercapacitor can be also used to overcome the electrochemical storage limits like its state of charge and maximum current. So, this thesis proposes the real time power control for a hybrid photovoltaic-battery-supercapacitor-diesel generator DC microgrid system, aiming to meet the load power demand with reliability and stabilizing the DC bus voltage. Both simulation and experimental results show that the designed control strategy improves the DC microgrid dynamic and static performances under different operating conditions. Furthermore, in order to minimize the diesel generator energy cost, the fuel cost and fuel consumption are analysed through several experimental tests. Therefore, the optimal value of its power generation is deduced and applied in a newly proposed energy management strategy. This strategy can achieve the goal of maximizing the utilization of photovoltaic energy and taking into account the slow start-up characteristic and energy cost of diesel generator. Both simulation and experimental studies are carried out by using the real photovoltaic data to illustrate the performance and the behavior of the hybrid system. The obtained results verify the effectiveness of this strategy. Furthermore, the comparison with the previous energy management strategy, in which the diesel generator energy cost is not considered, demonstrates that the newly proposed energy management strategy can reduce the total cost of the hybrid DC power system.

This study reviewed first the development of race result in canoe sprint during the past decades. The race results of MK1-1000 and WK1-500 have increased 32.5 % and 42.1 %, respectively, a corresponding 5.0 % and 6.5 % increase in each decade. The development of race results in canoe sprint during the past decades resulted from the contributions of various aspects. The recruitment of taller and stronger athletes improved the physiological capacity of paddlers. Direct investigation on energy contribution in canoe sprint enhanced the emphasis on aerobic capacity and aerobic endurance training. Advancement of equipment design improved the efficiency of paddling. Physiological and biomechanical diagnostics in canoe sprint led to a more scientific way of training. Additionally, other aspects might also have contributed to the development of race results during the past decades. For example, the establishment of national team after World War II provided the possibility of systematic training, and the use of drugs in the last century accelerated the development of race results in that period. Recent investigations on energetics in high-intensity exercises demonstrated an underestimate of WAER % in the table provided by some textbooks since the 1960s. An exponential correlation between WAER % and the duration of high-intensity exercises was concluded from summarizing most of the relevant reports, including reports with different methods of energy calculation. However, when reports with the MAOD and Pcr-La-O2 methods were summarized separately, a greater overestimate of WAER % from MAOD was found compared to those from Pcr-La-O2, which was in line with the critical reports on MAOD. Because of the lack of investigation of the validity of the comparisons between MAOD and Pcr-La-O2, it is still not clear which method can generate more accurate results and which method is more reliable. With regard to kayaking, a range of variation in WAER % was observed. Many factors might contribute to the variation of WAER % in kayaking. Therefore, the methods utilized to calculate the energy contributions, different paddling conditions, and the level of performance were investigated in kayaking. The findings indicated that the method utilized to calculate the energy contributions in kayaking, rather than paddling condition and performance level of paddlers, might be the possible factor associated with WAER %. Some other possible factors associated with WAER % still need to be further investigated in the future. After verifying the dependence of WAER % on the method of energy calculation, but not on paddling condition and performance level of paddlers, energy contributions of kayaking were investigated for the three racing distances on a kayak ergometer with junior paddlers. Energetic profiles in kayaking varied with paddling distances. At 500 m and 1000 m the aerobic system was dominant (with WAER % of 57.8 % and 76.2 %), whereas at 200 m the anaerobic system was dominant (with WAER % of 31.1-32.4 %). Muscular volume seemed to have an influence on absolute energy productions. The anaerobic alactic system determined the performance during the first 5 to 10 s. The anaerobic lactic system probably played a dominant role during the period from the 5th-10th s to 30th-40th s. The aerobic system could dominate the energy contribution after 30–40 s. This energetic profile in kayaking could provide physiological support for developing the training philosophy in these three distances. Additionally, the method introduced by Beneke et al. seemed to be a valid method to calculate the energy contributions in maximal kayaking. Energy contributions in canoeing were similar to those in kayaking. The relative energy contributions on open water canoeing were 75.3 ± 2.8 % of aerobic, 11.5 ± 1.9 % of anaerobic lactic, and 13.2 ± 1.9 % of anaerobic alactic at maximal speed of simulated 1000 m. Further, the C of canoeing seemed also to be similar to the reported findings in kayaking, with a function of y = 0.0242 * x2.1225. Training programs could be designed similarly for kayaking and canoeing with regard to energetic profile. In order to extend the findings on energetics in canoe sprint to other exercises, energy contributions in kayaking, canoeing, running, cycling, as well as arm cranking were compared with the same duration. Results indicated that WAER % during maximal exercises with the same duration seemed to be independent of movement patterns, given similar VO2 kinetics during the maximal exertion. The exponential relationship between WAER % and duration in maximal exercises could be supported by excluding the influence from movement patterns. Additionally, MLSS in kayaking was investigated. The blood lactate value of MLSS was found to be 5.4 mM in kayaking, which could expand the knowledge of MLSS in different locomotion. The MLSS in kayaking might be attributed to the involved muscle mass in this locomotion, which could result in a certain level of lactate removal, and allow a certain level of equilibrium between lactate production and removal. LT5, instead of LT4, was recommended for diagnostics in kayaking, given an incremental test as used in this study.

The functionality to both charge and discharge energy from and to the power grid to a Battery Electric Vehicle (BEV) is referred to as Vehicle-to-Grid (V2G). This allows the customer to buy energy when the spot price is low and sell energy when the price is high to make a profit, called energy arbitrage. However, when the battery is charging, discharging, or idling for storage, battery degradation occurs due to chemical properties and reactions. This thesis developed a mathematical optimisation model in Python, using the modelling language Pyomo. Mathematical equations are used to integrate energy arbitrage and degradation data to reduce the total cost in terms of degradation and energy by finding an optimised charge and discharge pattern. The model allows different user scenarios to be analysed by changing inputs such as charger power, battery cost or daily driving distance. When using V2G technology, the State-of-Charge (SoC) level of BEVs battery packs can be adjusted to find SoC levels which minimise the battery degradation, while allowing the user to make a profit from energy arbitrage. The result shows that the V2G charging protocol, compared to protocols without a bidirectional charger could be beneficial for the simulated time periods, by both reducing degradation and the total energy cost. The results also indicate that the degradation cost of the battery is often the determining factor in the decision of when to charge or discharge, i.e., the substantial cost-saving strategy is to control the storage and cycle degradation to reduce the total degradation, rather than controlling the energy arbitrage. The model and the result of this thesis can be used by car manufacturers to learn more about how battery cell types behave in V2G mode and influence further work on V2G control.

No description available.

This dissertation attempts to integrate the household and children more fluidly into measures of well-being in the past. In part one, I develop a Monte Carlo simulation to test some of the assumptions of Allen’s welfare ratio methodology. These included his assumptions that family size was constant over time, that there were no female-headed households and that women and children did not participate in the labour force. After all of the adjustments, it appears that Allen’s welfare ratios underestimate the welfare ratios of a demographically representative group of families, especially if women and children’s labour force participation is included. However, the predicted distributions also highlight the struggles of agricultural labourers, who are given separate consideration. Even the average agricultural labourers’ family with women and children working would have had to rely of self- provisioning, gleaning, poor relief or the extension of the working year to make ends meet at the poorest point in their family life cycle. Part two adjusts Floud et al.’s estimates of calorie availability in the English economy from 1700 to 1909 for the costs of digestion, pregnancy and lactation. Taken together, these three additional costs reduced the amount calories available by around 15 per cent in 1700 but only by 5 per cent in 1909 because of the changing composition of the English diet. Part three presents a new adaptive framework for studying changes in children’s growth patterns over time and a new methodology, longitudinal growth studies, for measuring gender disparities in health in the past. An adaptive framework for understanding growth provides a more parsimonious explanation for the vast catch-up growth achieved by slave children in the antebellum American South. The slave children were only able to achieve this catch-up growth because they were programmed for a tall height trajectory by relatively good conditions in utero. Finally, impoverished girls experienced greater catch-up growth than boys in two schools in late-nineteenth century Boston, USA and early-twentieth century London, suggesting that girls were deprived relative to boys before entering these institutions.

Thèse effectuée en cotutelle (Université de Poitiers, France) / Introduction – Le vieillissement est associé à une augmentation du coût énergétique de la locomotion (CE) et à une diminution de la consommation maximale d’oxygène (V ̇O2max). De plus, les qualités neuromusculaires semblent aussi compromises chez les aînés. Ces phénomènes contribuent au ralentissement de la vitesse de marche spontanée et peuvent altérer la qualité de vie. En plus de ces qualités physiques, il est reconnu que les fonctions cognitives déterminent la mobilité. En effet, la capacité de combiner une tâche motrice (locomotion) à une tâche cognitive est associée à une réduction du risque de chuter. Objectif – L’objectif de cette thèse était de préciser les relations entre la mobilité, la condition physique et les fonctions cognitives dans une perspective de vieillissement sain. Résultats – Une étude méthodologique a été présentée pour spécifier les modalités d’évaluation du CE. Les résultats ont démontré que le CE est plus élevé lorsque la mesure est effectuée sur tapis roulant en comparaison avec une mesure directement au sol. Il a aussi été suggéré que le niveau d’assocation entre les deux modalités était insatisfaisant. Ainsi, lorsque la mesure sur tapis roulant est nécessaire, elle doit idéalement être accompagnée d’un test de marche au sol pour améliorer la validité externe. Ensuite, une approche transversale a été utilisée afin de préciser les déterminants de la condition physique associés à une meilleure mobilité et à un profil cognitif favorable. En plus de la V ̇O2max, une force élevée caractérisait les individus qui présentaient les meilleures performances dans une tâche de flexibilité cognitive. Il a aussi été démontré que des tests cliniques comme le Timed up and go et la vitesse maximale de marche sur 10 mètres pouvaient être utilisés pour discriminer les individus qui présentent les profils physiques et cognitifs les moins favorables. Enfin, une étude d’intervention a été proposée afin de vérifier si un entraînement de l’aptitude aérobie et de la force visant une augmentation de la V ̇O2max et la réduction du CE allait permettre de meilleures performances cognitives lors d’une double tâche (motrice et cognitive). Les résultats démontrent, malgré que ce type d’intervention semble favoriser de meilleures performances en inhibition, qu’un programme visant le développement des habiletés motrices globales est aussi efficace pour induire des améliorations en inhibition. Conclusion – Ces résultats confirment la pertinence de l’évaluation d’un ensemble de déterminants de la condition physique allant de l’aptitude aérobie aux qualités neuromsuculaires dans le suivi de la santé physique et cognitive des aînés. Des tests cliniques accessibles sont proposés afin de contribuer à un premier diagnostic physique et cognitif qui saura être confirmé par des mesures plus exhaustives. Par ailleurs, il est aussi suggéré que plusieurs interventions peuvent être proposées pour améliorer les fonctions cognitives. Malgré que l’entraînement de l’aptitude aérobie et des qualités de force soit le seul à induire des améliorations de la condition physique, il n’en demeure pas moins qu’un programme de développement des habiletés motrices puisse représenter une avenue intéressante dans le cas où les individus étaient au préalable très sédentaires. / Introduction – Aging is characterized by an increase in the energy cost of locomotion (ECL) and a decrease in maximal oxygen uptake (V ̇O2max). It also appears that neuromuscular performances are jeopardized in older adults. Taken together, these phenomena contribute to a slower gait speed, which could reduce quality of life. Moreover, it was shown that cognition was also a key determinant of mobility performances. Indeed, the ability to execute simultaneously two tasks (motor and cognitive) was associated with a lower risk of falling. Objective – The objective of this thesis was to specify the relationships between mobility, physical fitness and cognition in a perspective of healthy aging. Results – A study was conducted to clarify the ECL testing methods. Results demonstrate a higher ECL when testing occurs on a treadmill in comparison to an overground measure. Moreover, it was shown that the association level was unsatisfying between the two testing modes. It was concluded that, when a treadmill testing is necessary, a complementary functional walking test should be implemented to increase external validity in data interpretation. Afterwards, a cross-sectional analysis was used to determine the physical fitness factors associated with better cognitive and mobility performances. Along with V ̇O2max, higher levels of strength were related to the fastest individuals both in mobility and in cognitive flexibility. Also, it was shown that clinical tests like the Timed up and go and the maximal walking speed over 10 meters were able to distinguish individuals with poorer physical fitness and cognitive performances. Finally, an intervention study was proposed to verify the effects of a combined aerobic and strength training program on physical fitness and cognition. It was hypothesized that reducing the ECL and increasing V ̇O2max would induce better cognitive performances during a dual-task (motor and cognitive). Results showed that this intervention improved inhibition performances. However, data also suggested that a training program aiming for gross motor abilities development was as efficient as the fitness training intervention to improve inhibition. Conclusion – Taken together, these results confirm the relevance of a complete physical fitness assessment in an healthy aging perspective including both aerobic and neuromuscular components. Convenient clinical tests are also proposed to establish a first physical and cognitive diagnosis, which will be confirmed by subsequent more exhaustive tests. Moreover, it was suggested that many interventions were leading to better cognitive performances. Along with aerobic and strength training which is a privileged method to improve physical fitness, programs aiming for gross motor abilities development could also be implemented, especially in a context where individuals are particularly sedentary.

La problématique du port de charges par l’Homme est l’objet de questionnements scientifiques depuis plus d’un siècle, notamment dans les armées où les soldats doivent remplir des objectifs opérationnels tout en emportant des équipements lourds, distribués de façon complexe autour de leur corps, et cela au cours d’efforts allant de quelques heures à plusieurs jours. Aussi, avec le développement des nouvelles technologies et l’arrivée des systèmes fantassins futurs sur le marché de la défense, cette problématique continue de se complexifier puisque la masse totale emportée tend encore à croître. Objectif général : Le but de ce travail de thèse était d’étudier l’impact biomécanique, métabolique et neuromusculaire du port d’un système fantassin moderne chez le soldat expérimenté. Plus précisément, une première recherche a été menée pour caractériser les effets aigus du port d’un tel équipement sur la biomécanique et le coût métabolique de la marche. Puis, une seconde recherche a été consacrée à l’étude des conséquences neuromusculaires et locomotrices d’une mission militaire (simulation sur le terrain) de durée « extrême » réalisée avec ce système fantassin moderne. Première partie : L’analyse de la marche sur tapis roulant dynamométrique a permis de montrer que le port du système fantassin en configurations de « combat » et de « marche d’approche » (principales configurations du théâtre militaire, représentant respectivement ~30 % et ~50 % de la masse corporelle des sujets) altérait le pattern spatio-temporel par rapport à la marche sans charge. Par ailleurs, le travail mécanique appliqué au centre de masse et le coût métabolique de la marche augmentaient parallèlement lors du port des deux configurations du système fantassin, ce qui résultait en un maintien du rendement locomoteur constant dans toutes les conditions testées. Le mécanisme de transfert d’énergie en pendule inversé (méthode Cavagna), permettant de minimiser les coûts mécanique et métabolique de transport, était également similaire dans toutes les conditions avec et sans charge. Enfin, bien que complexement organisés autour du corps du soldat, les équipements militaires n’induisaient pas d’effets mécaniques et métaboliques sensiblement plus importants que ceux rapportés lors du port de masses positionnées symétriquement autour de la taille ; ce mode de portage étant pourtant considéré comme l’un des plus optimisés, abstraction faite des techniques de portage sur la tête inadaptées au contexte militaire. Deuxième partie : La réalisation d’une mission simulée, incluant 21 h d’activités militaires sur le terrain et le port constant d’un système fantassin, résultait en une fatigue neuromusculaire (mesure des forces, électrostimulation et EMG) relativement modérée des muscles locomoteurs extenseurs du genou et fléchisseurs plantaires chez les soldats expérimentés inclus dans ce travail. Les origines de cette fatigue neuromusculaire étaient essentiellement périphériques, mais s’accompagnaient d’une fatigue subjective importante. Enfin, la réalisation de la mission, et donc la fatigue des muscles locomoteurs notamment associée à cette dernière, n’affectait pas sensiblement les paramètres mécaniques et métaboliques de la marche. Conclusion générale : Ce travail rapporte les premières données relatives aux effets biomécaniques, métaboliques et neuromusculaires du port d’un système fantassin moderne chez le soldat expérimenté, et ce par le biais d’une simulation opérationnelle visant à reproduire les conditions militaires / For more than a century, load carriage has been the source of many scientific questions and researches, especially in armies in which soldiers have to reach operational goals while carrying heavy loads complexly distributed around their body and this for some hours to several days. Moreover, with the development of new technologies and the advent of land warfare systems, this issue is becoming more complex since load continues to increase. General purpose: The aim of the present work was to investigate the biomechanical, metabolic and neuromuscular constraints imposed by the carriage of a modern land warfare system in experienced soldiers. Specifically, a first study was conducted to characterize the acute effects of land warfare equipments carriage on the biomechanics and energetics of walking. Moreover, a second research was performed to investigate the neuromuscular and locomotor consequences of an extreme-duration simulated military mission (performed in the field) involving the carriage of these specific equipments. First part: Walking analyses performed on an instrumented treadmill showed that the carriage of a land warfare system in two configurations made for “Battles” and “Road marches” (main configuration used in the field representing ~30% and ~50% of subject’s body mass, respectively) altered the walking pattern. Moreover, the mechanical work applied to the center of mass increased in parallel with the energy cost of walking during Battle and Road March configurations carriage, as shown by the constant locomotor efficiency in the three tested conditions. The inverted pendulum-like energy exchange (Cavagna’s standard method), allowing mechanical/metabolic energy saving, was also constant in all the unloaded and loaded conditions. Finally, even if the load was complexly distributed around soldiers’ body with this warfare system, this did not induce greater effects than those reported with loads carried symmetrically around the waist and close to the center of mass; which has yet been hypothesized to be the optimal method of load carriage from both mechanical and metabolic standpoints (except “original” methods such as head carrying that cannot be used in the military theater). Second part: Performing a 21-h simulated military mission with heavy land warfare system carriage induced relatively moderate neuromuscular alterations (isometric force measurement with electrical stimulation and EMG) for both knee extensors and plantar flexors locomotor muscle groups in experienced soldiers. Moreover, the origin of fatigue was essentially peripheral for both muscle groups but was concomitant with a large sensation of fatigue. Finally, the simulated mission, and notably the associated locomotor muscles fatigue, did not alter walking biomechanics and energetics. General conclusion: These results bring the first insight into the biomechanical, metabolic and neuromuscular consequences of modern land warfare system carriage in experienced soldiers, and this by a real-world approach aiming at simulating the military theater conditions

Le métabolisme est la somme des réactions chimiques dans un organisme. L’énergie leur permet d’effectuer la biosynthèse, le maintien des fonctions vitales et l’activité physique. Si l’énergie est transformée en chaleur nous pouvons déterminer le taux de transformation d’énergie chimique en taux métabolique (TM) et nous pouvons donc calculer les besoins énergétiques d’un animal. Le principal objectif de ce travail est d’évaluer le métabolisme énergétique chez la punaise hématophage Rhodnius prolixus. Nos résultats montrent, que le TM de l’alimentation chez R. prolixus atteint jusqu’à 17 fois le TM au repos. Le quotient respiratoire est de 0,83 pendant le repos et 0,52 pendant l’alimentation. De plus, la désactivation des protéines permettant gérer le choc thermique, diminuent le TM pendant la digestion. Le coût énergétique (CE) de la marche peut atteindre jusqu’à 1,7 fois le CE pendant le repos. Finalement, le CE de la production d’un oeuf chez R. prolixus est approximativement de 11,7 J. L’ensemble de nos résultats apportent des outils qui fournissent des éléments nécessaires pour mieux contrôler la transmission de maladies vectorielles. / Metabolism is the sum of all the chemical reactions in an organism. Energy uptake allows animals to perform biosynthesis, maintenance, and external work. If the energy produced is converted into heat, we can estimate the energy metabolism as the rate of conversion of chemical energy into metabolic rate (MR), which allows the calculation of energy requirements. The main objective of this work is to assess the metabolic cost of physiological work in the blood-sucking bug Rhodnius prolixus. Our results show that feeding is costly. The MR during feeding in R. prolixus reaches up to 17 times the MR during rest. The mean respiratory quotient is 0.83 during rest and 0.52 during feeding. We showed that the deactivation of Heat Shock Proteins on R. prolixus, causes a diminution of MR during digestion. Then, we showed that the energy cost (EC) during walking can reach up to 1.7 times the EC during rest. Finally, we found that the EC of production of one egg of R. prolixus was 11.7 J. All our results provide tools to a better understanding of biology and ecology of an hematophagous insect to provide the necessary elements to better control of transmission of vector-borne diseases.

La myopathie némaline (MN) est une maladie neuromusculaire rare représentant la forme la plus répandue de myopathie congénitale. Elle est caractérisée par une faiblesse musculaire et la présence de structures en forme de bâtonnets au sein de la fibre musculaire squelettique. Au cours des dix dernières années, le développement de plusieurs modèles murins de MN a permis de mettre en évidence in vitro plusieurs mécanismes physiopatholgiques responsables, au moins en partie, de la faiblesse musculaire associée à la MN. Ce projet était donc dédié à la caractérisation phénotypique in vivo de la fonction musculaire squelettique de quatre modèles murins de MN reproduisant des formes humaines légères et sévères. A l’aide d’un dispositif expérimental précédemment développé au laboratoire, la fonction musculaire squelettique a été évaluée de manière strictement noninvasive à l’aide des techniques d’imagerie de résonance magnétique (IRM) multimodale (Dixon, T2, imagerie du tenseur de diffusion (DTI)) et de spectroscopie de résonance magnétique du phosphore 31 (SRM-P31) associées à des mesures mécaniques. Nous avons démontré que les altérations observées in vivo ne sont pas nécessairement similaires à celles rapportées in vitro. La SRM-P31 nous a permis de mettre en évidence des altérations métaboliques uniquement pour les formes sévères. Une absence d’infiltration intramusculaire de tissu adipeux a également été observée à l’aide de l’IRM Dixon. Enfin, les techniques d’IRM T2 et de DTI nous ont permis de déterminer des biomarqueurs noninvasifs et quantitatifs qui pourraient, à terme, permettre d’évaluer la sévérité et/ou la progression de la maladie chez les patients atteints de MN. / Nemaline myopathy (NM) is the most common of the non-dystrophic congenital myopathies and is characterized by muscle weakness and accumulation of an electron dense material (rods) within the sarcomeric units. Over the past decade, the generation of mouse models of NM allowed to identify in vitro several physiopathological mechanisms involved, at least in part, in muscle weakness in NM. This project has been devoted to the in vivo phenotypic characterization of the skeletal muscle function of mouse models mimicking mild and severe human forms of NM. Skeletal muscle function was assessed on four mouse models of NM with an original and strictly noninvasive experimental setup designed and built in our laboratory allowing 31P-magnetic resonance spectroscopy (31P-MRS) and multimodal magnetic resonance imaging (MRI) investigations, including Dixon, T2 mapping and Diffusion Tensor Imaging (DTI). Our results suggest that in vitro alterations did not necessarily translate into similar changes in vivo. 31P-MRS illustrated an altered energy metabolism only for the mouse models mimicking severe form of NM. Our Dixon MRI investigations showed that fatty infiltration was negligible. Finally, T2 maps and DTI experiments provided relevant noninvasive and quantitative biomarkers for monitoring the severity and/or the progression of NM in patients.

No description available.