Quelle R&D Mener pour le Développement Des Réseaux D'énergie De Demain ? Les Propositions de L'ancre en 2015

Feuille de route sur les réseaux électriques et stockage élaborée par le GP10 Réseaux et Stockages de l'Energie de l'ANCRECette feuille de route concerne les réseaux d’énergie électrique, de chaleur et de froid, les réseaux de gaz (hydrogène, gaz naturel), leurs stockages associés, ainsi que leurs couplages à venir dans le cadre de la transition énergétique et des évolutionsqui l’accompagneront, que ce soit sur les modes de production d’énergie ou sur l’évolution des usages.Le focus est porté sur les réseaux électriques qui seront les premiers impactés par cette transition énergétique. Hormisquelques éléments très spécifiques aux réseaux électriques (et qui seront notés dans le texte par une couleur différente)il est à souligner que la quasi-totalité des considérations et axes de R&D évoqués pour les réseauxélectriques et le développement de leur « intelligence » et/ou de leur flexibilité s’appliquentégalement aux autres réseaux d’énergie. Par ailleurs, si le groupe programmatique« Réseaux et Stockage » de l’ANCRE (GP10) s’est largement appuyé sur les nombreuses feuilles de route émises tant au niveau national, dont celles de l’ADEME, qu’européen, il a également souhaité s’en démarquer en insistantlargement et en détaillant les recherches scientifiques et technologiques à mener face aux verrous actuellement identifiés

Exhaustive analysis of BH4 and dopamine biosynthesis genes in patients with Dopa-responsive dystonia

Dopa-responsive dystonia is a childhood-onset dystonic disorder, characterized by a dramatic response to low dose of l-Dopa. Dopa-responsive dystonia is mostly caused by autosomal dominant mutations in the GCH1 gene (GTP cyclohydrolase1) and more rarely by autosomal recessive mutations in the TH (tyrosine hydroxylase) or SPR (sepiapterin reductase) genes. In addition, mutations in the PARK2 gene (parkin) which causes autosomal recessive juvenile parkinsonism may present as Dopa-responsive dystonia. In order to evaluate the relative frequency of the mutations in these genes, but also in the genes involved in the biosynthesis and recycling of BH4, and to evaluate the associated clinical spectrum, we have studied a large series of index patients (n = 64) with Dopa-responsive dystonia, in whom dystonia improved by at least 50% after l-Dopa treatment. Fifty seven of these patients were classified as pure Dopa-responsive dystonia and seven as Dopa-responsive dystonia-plus syndromes. All patients were screened for point mutations and large rearrangements in the GCH1 gene, followed by sequencing of the TH and SPR genes, then PTS (pyruvoyl tetrahydropterin synthase), PCBD (pterin-4a-carbinolamine dehydratase), QDPR (dihydropteridin reductase) and PARK2 (parkin) genes. We identified 34 different heterozygous point mutations in 40 patients, and six different large deletions in seven patients in the GCH1 gene. Except for one patient with mental retardation and a large deletion of 2.3 Mb encompassing 10 genes, all patients had stereotyped clinical features, characterized by pure Dopa-responsive dystonia with onset in the lower limbs and an excellent response to low doses of l-Dopa. Dystonia started in the first decade of life in 40 patients (85%) and before the age of 1 year in one patient (2.2%). Three of the 17 negative GCH1 patients had mutations in the TH gene, two in the SPR gene and one in the PARK2 gene. No mutations in the three genes involved in the biosynthesis and recycling of BH4 were identified. The clinical presentations of patients with mutations in TH and SPR genes were strikingly more complex, characterized by mental retardation, oculogyric crises and parkinsonism and they were all classified as Dopa-responsive dystonia-plus syndromes. Patient with mutation in the PARK2 gene had Dopa-responsive dystonia with a good improvement with l-Dopa, similar to Dopa-responsive dystonia secondary to GCH1 mutations. Although the yield of mutations exceeds 80% in pure Dopa-responsive dystonia and Dopa-responsive dystonia-plus syndromes groups, the genes involved are clearly different: GCH1 in the former and TH and SPR in the late

Training in hypoxia fails to further enhance endurance performance and lactate clearance in well-trained men and impairs glucose metabolism during prolonged exercise.

The aim of this study was to investigate the synergistic effects of endurance training and hypoxia on endurance performance in normoxic and hypoxic conditions (approximately 3000 m above sea level) as well as on lactate and glucose metabolism during prolonged exercise. For this purpose, 14 well-trained cyclists performed 12 training sessions in conditions of normobaric hypoxia (HYP group, n = 7) or normoxia (NOR group, n = 7) over 4 weeks. Before and after training, lactate and glucose turnover rates were measured by infusion of exogenous lactate and stable isotope tracers. Endurance performance was assessed during incremental tests performed in normoxia and hypoxia and a 40 km time trial performed in normoxia. After training, performance was similarly and significantly improved in the NOR and HYP groups (training, P < 0.001) in normoxic conditions. No further effect of hypoxic training was found on markers of endurance performance in hypoxia (training x hypoxia interaction, n.s.). In addition, training and hypoxia had no significant effect on lactate turnover rate. In contrast, there was a significant interaction of training and hypoxia (P < 0.05) on glucose metabolism, as follows: plasma insulin and glucose concentrations were significantly increased; glucose metabolic clearance rate was decreased; and the insulin to glucagon ratio was increased after training in the HYP group. In conclusion, our results show that, compared with training in normoxia, training in hypoxia has no further effect on endurance performance in both normoxic and hypoxic conditions or on lactate metabolic clearance rate. Additionally, these findings suggest that training in hypoxia impairs blood glucose regulation in endurance-trained subjects during exercise

Les moulins à tan et marteaux à battre le cuir de Bretteville-sur-Laize, Fresney-le-Puceux, Gouvix (1874-1920)

Ponsot Philippe. Les moulins à tan et marteaux à battre le cuir de Bretteville-sur-Laize, Fresney-le-Puceux, Gouvix (1874-1920). In: Annales de Normandie, 34ᵉ année, n°2, 1984. pp. 171-189

Douvres-la-Délivrande (1650-1792) — Une inversion démographique ?

Après un rapide panorama de la société de Douvres aux XVIIe et XVIIIe siècles, l'auteur étudie les structures démographiques : mariages, naissances, mortalité, étrangers, migrations. Les conclusions auxquelles il aboutit donnent une figure originale à l'évolution de la population de ce bourg de la plaine de Caen, dont le pèlerinage de la Délivrande constitue l'activité principale. Le doublement de la population au cours de la seconde moitié du XVIIe siècle se poursuit par un accroissement plus lent pendant le demi-siècle suivant que marquent des baisses de natalité et de nuptialité en même temps que de fortes poussées de mortalité épidémique. La croissance rapide reprend à partir de 1760 en contraste avec la stabilisation ou le déclin de nombreux villages à la même époque.Ponsot Philippe. Douvres-la-Délivrande (1650-1792) — Une inversion démographique ?. In: Annales de Normandie, 14ᵉ année, n°4, 1964. pp. 455-477

La fabrication du cuir fort par tannage lent, dans la vallée de la Laize, de 1775 à 1944 : étude d'un patrimoine industriel

Ponsot Philippe. La fabrication du cuir fort par tannage lent, dans la vallée de la Laize, de 1775 à 1944 : étude d'un patrimoine industriel. In: Cahier des Annales de Normandie n°24, 1992. Recueil d'études offert à Gabriel Désert. pp. 271-285

Les troubles de la deglutition et de l'alimentation chez l'enfant et l'adulte polyhandicapes Le Kremlin Bicetre, 28-29 septembre 2000, resumes

Available from INIST (FR), Document Supply Service, under shelf-number : Y 33399 / INIST-CNRS - Institut de l'Information Scientifique et TechniqueSIGLEAssistance Publique - Hopitaux de Paris, 75 - Paris (France). Mission HandicapsFRFranc

South America and new financial architecture

International audienceThe new monetary and financial architecture in South America rests on two important premises: a financial argument built around the creation of a new development bank, the Banco del Sur, and a monetary argument, founded on the notion of monetary regional integration, independent of the use of the U.S. dollar. This architecture contrasts with the main model of regional monetary integration that consists of two possible solutions: full monetary union or dollarization. The second model of regional monetary integration, however, is more consistent with Keynes and Post Keynesians. It rests on the creation of a common currency—or a unit of account—that does not supersede the existence of national currencies. This currency would be used solely for settling interregional payments, much like Keynes's bancor

An Integrative Model Accounting for the Symptom Cluster Triggered After an Acoustic Shock

Acoustic shocks and traumas sometimes result in a cluster of debilitating symptoms, including tinnitus, hyperacusis, ear fullness and tension, dizziness, and pain in and outside the ear. The mechanisms underlying this large variety of symptoms remain elusive. In this article, we elaborate on the hypothesis that the tensor tympani muscle (TTM), the trigeminal nerve (TGN), and the trigeminal cervical complex (TCC) play a central role in generating these symptoms. We argue that TTM overuse (due to the acoustic shock), TTM overload (due to muscle tension), and ultimately, TTM injury (due to hypoxia and “energy crisis”) lead to inflammation, thereby activating the TGN, TCC, and cortex. The TCC is a crossroad structure integrating sensory inputs coming from the head–neck complex (including the middle ear) and projecting back to it. The multimodal integration of the TCC may then account for referred pain outside the ear when the middle ear is inflamed and activates the TGN. We believe that our model proposes a synthetic and explanatory framework to explain the phenomena occurring postacoustic shock and potentially also after other nonauditory causes. Indeed, due to the bidirectional properties of the TCC, musculoskeletal disorders in the region of the head–neck complex, including neck injury due to whiplash or temporomandibular disorders, may impact the middle ear, thereby leading to otic symptoms. This previously unavailable model type is experimentally testable and must be taken as a starting point for identifying the mechanisms responsible for this particular subtype of tinnitus and its associated symptoms