Najnowsze osiągnięcia turyzmu alpejskiego w aspekcie cyklu rozwoju

The article presents recent trends in the tourist economy in the Alpine region. With reference to the development cycle of the tourist product, proposed by Butler, the author claims that the Alpine tourist centres have already reached the maturity stage. Their further development requires active intervention in order to refresh the tourist product. Further in the article, a variety of tourist development strategies have been presented, which might improve the poor condition of many tourist centres in the region under discussion.W artykule przedstawiono najnowsze trendy w zakresie gospodarki turystycznej w regionie Alp. Autor, w nawiązaniu do przedstawionego przez Butlera cyklu rozwojowego produktu turystycznego, stwierdził, że alpejskie ośrodki turystyczne osiągnęły już etap dojrzałości. Dalszy ich rozwój wymaga podjęcia aktywnej interwencji, zmierzającej do odświeżenia produktu turystycznego. W dalszej części pracy zaprezentowano różne strategie rozwoju turystyki, mające na celu zmianę trudnej sytuacji wielu ośrodków turystycznych w omawianym regionie

La diversificación de la oferta turística en las estaciones de media montaña: el caso de la Chapelle en los Alpes franceses del Norte

La Chapelle is a small ski resort in the northern Alps, France. This paper explores the tourist activities offer diversification in mid-altitude mountain areas. The results show that: 1) despite the low altitude ski area (1000-1800 m), the commune’s policy is still heavily in favour of skiing; 2) Summer activities appear to be primarily developed by the ski lift group (mountain biking, devalkart, rollerherbe... thereby creating the need for ski lifts during the summer) rather than local public policy makers. Agritourism, rural and cultural activities appear neglected.Este trabajo estudia la diversificación de la oferta turística en la media montaña (el caso de La Chapelle, pequeña estación de esquí, Alpes del Norte, Francia). 1/ A pesar de la modesta altitud de la zona esquiable (1000-1800 m), La Chapelle apuesta fuertemente por el esquí. 2/ La oferta de verano parece estar más ligada a la organización que administra los telesillas (descenso en bicicletas de montaña, devalkart o rollerherbe que permite rentabilizar los telesillas en verano) que a las políticas públicas locales. El agroturismo, las actividades rurales o culturales no se tienen en cuenta

Nuclear factors involved in mitochondrial translation cause a subgroup of combined respiratory chain deficiency.

Mutations in several mitochondrial DNA and nuclear genes involved in mitochondrial protein synthesis have recently been reported in combined respiratory chain deficiency, indicating a generalized defect in mitochondrial translation. However, the number of patients with pathogenic mutations is small, implying that nuclear defects of mitochondrial translation are either underdiagnosed or intrauterine lethal. No comprehensive studies have been reported on large cohorts of patients with combined respiratory chain deficiency addressing the role of nuclear genes affecting mitochondrial protein synthesis to date. We investigated a cohort of 52 patients with combined respiratory chain deficiency without causative mitochondrial DNA mutations, rearrangements or depletion, to determine whether a defect in mitochondrial translation defines the pathomechanism of their clinical disease. We followed a combined approach of sequencing known nuclear genes involved in mitochondrial protein synthesis (EFG1, EFTu, EFTs, MRPS16, TRMU), as well as performing in vitro functional studies in 22 patient cell lines. The majority of our patients were children (<15 years), with an early onset of symptoms <1 year of age (65%). The most frequent clinical presentation was mitochondrial encephalomyopathy (63%); however, a number of patients showed cardiomyopathy (33%), isolated myopathy (15%) or hepatopathy (13%). Genomic sequencing revealed compound heterozygous mutations in the mitochondrial transfer ribonucleic acid modifying factor (TRMU) in a single patient only, presenting with early onset, reversible liver disease. No pathogenic mutation was detected in any of the remaining 51 patients in the other genes analysed. In vivo labelling of mitochondrial polypeptides in 22 patient cell lines showed overall (three patients) or selective (four patients) defects of mitochondrial translation. Immunoblotting for mitochondrial proteins revealed decreased steady state levels of proteins in some patients, but normal or increased levels in others, indicating a possible compensatory mechanism. In summary, candidate gene sequencing in this group of patients has a very low detection rate (1/52), although in vivo labelling of mitochondrial translation in 22 patient cell lines indicate that a nuclear defect affecting mitochondrial protein synthesis is responsible for about one-third of combined respiratory chain deficiencies (7/22). In the remaining patients, the impaired respiratory chain activity is most likely the consequence of several different events downstream of mitochondrial translation. Clinical classification of patients with biochemical analysis, genetic testing and, more importantly, in vivo labelling and immunoblotting of mitochondrial proteins show incoherent results, but a systematic review of these data in more patients may reveal underlying mechanisms, and facilitate the identification of novel factors involved in combined respiratory chain deficiency

Biological Earth observation with animal sensors

Space-based tracking technology using low-cost miniature tags is now delivering data on fine-scale animal movement at near-global scale. Linked with remotely sensed environmental data, this offers a biological lens on habitat integrity and connectivity for conservation and human health; a global network of animal sentinels of environmen-tal change

A. Suchet et A. El Akari (dir.), Développement du sport et dynamique des territoires : expériences internationales comparées

Cet ouvrage est le fruit des travaux de la 8ème Biennale de l’Association Francophone pour la Recherche en Activités Physiques et Sportives (AFRAPS) qui s’est tenue au Maroc en avril 2018. Au total, suite à une courte partie introductive, le cœur de ce volume est composé de 26 contributions, réparties entre quatre sections : Sport et tourisme dans l’organisation des territoires ; configurations nationales et politiques sportives ; développement du sport, éducation et intégration sociale ; et ..

Mathis Stock et al., Les enjeux contemporains du tourisme : une approche géographique

Reviewing a book on « Contemporary issues in tourism » at a time when the prevalence of Covid-19 throughout the world is causing widespread disruption to tourist activities and an almost complete halt to international travel is not without its challenges. This is particularly the case as this text was written before the emergence of these problems and quite naturally considers many of the numerous aspects of the growth and development of tourism and its global spread. However, this in no way ..

Yvette Veyret et Paul Arnould (dir.), Atlas des développements durables

Traiter les caractéristiques et les enjeux du développement durable en moins de 90 pages est un défi ambitieux mais il a été largement relevé dans l’Atlas des développements durables proposé par Yvette Veyret et Paul Arnould. Plus d’une trentaine d’auteurs, tous des spécialistes des questions élaborées, offrent des analyses concises et intéressantes, utilisant souvent des études de cas détaillés. Les textes sont accompagnés de schémas et de cartes clairs et pertinents, tous en couleur, créant..

Mutations et inerties spatiales dans le Royaume-Uni d'aujourd'hui

Le Royaume-Uni n’est pas à l’honneur dans la littérature géographique française. Pourtant, notre voisin d’Outre-Manche ne manque pas d’intérêt pour le chercheur. « Exotique », tour à tour avant-gardiste et conservatrice, vue de France, Albion est souvent trop perfide pour se laisser appréhender. Cependant, aussi différent que le Royaume-Uni puisse être de la France, il se voit imposées les mêmes opportunités et contraintes liées à la mondialisation. Ce sont plutôt les traductions sociales et ..