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排序方式: 共有734条查询结果,搜索用时 531 毫秒
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Jeanne Amiel Nadine Gigarel Alexandra Benacki Paule Benit Isabelle Valnot Béatrice Parfait Jurgen-Christoph Von Kleist-Retzow Valérie Raclin Smaïl Hadj-Rabia Yves Dumez Pierre Rustin Jean-Paul Bonnefont Arnold Munnich Agnès Rötig 《黑龙江环境通报》2001,21(7):602-604
Respiratory chain deficiency (RCD) is responsible for a clinically heterogeneous group of early-onset untreatable disorders. Enzymological prenatal diagnosis (PD) can only be offered to a fraction of families. Moreover, due to the two-fold genetic origin of the respiratory chain (nuclear and mitochondrial DNA) and owing to the large number of nuclear genes involved in the respiratory chain assembly, maintenance and functioning, the identification of the disease causing gene in a given family remains challenging. Here, we report on PD of RCD by direct screening of NDUFV1, SDH-Fp, SCO1 and SURF1 mutations in five unrelated families with complex I, II and IV deficiency, respectively. The identification of the disease-causing gene in a given family with RCD is a major issue to provide both adequate genetic counselling and early, reliable PD. Copyright © 2001 John Wiley & Sons, Ltd. 相似文献
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Bénédicte Rulleau Hélène Rey-Valette Cécile Hérivaux 《Journal of Environmental Planning and Management》2015,58(3):482-494
Beach protection has become a major issue in reducing coastal risks (erosion and flooding). It is thus advisable to study residents’ preferences for mitigation strategies. Willingness-to-pay (WTP) by permanent and secondary residents for flood protection provided to properties by Languedoc-Roussillon beaches (French Mediterranean coast) is investigated by a contingent valuation study. Results show that WTP is more influenced by risk perception variables than by socio-economic ones. The WTP is then extrapolated on the basis of different adaptation strategies (laissez-faire, managed retreat, denial etc.) which provided information about expected damage associated with sea level rise at the 2100 time horizon. 相似文献
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Akcha Farida Barranger Audrey Bachère Evelyne 《Environmental science and pollution research international》2021,28(7):8266-8280
Environmental Science and Pollution Research - Recently, research has contributed to better knowledge on the occurrence of pesticides in coastal water by identifying frequently detected substances,... 相似文献
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Anne Ganteaume Andrea Camia Marielle Jappiot Jesus San-Miguel-Ayanz Marlène Long-Fournel Corinne Lampin 《Environmental management》2013,51(3):651-662
Knowledge of the causes of forest fires, and of the main driving factors of ignition, is an indispensable step towards effective fire prevention policies. This study analyses the factors driving forest fire ignition in the Mediterranean region including the most common human and environmental factors used for modelling in the European context. Fire ignition factors are compared to spatial and temporal variations of fire occurrence in the region, then are compared to results obtained in other areas of the world, with a special focus on North America (US and Canada) where a significant number of studies has been carried out on this topic. The causes of forest fires are varied and their distribution differs among countries, but may also differ spatially and temporally within the same country. In Europe, and especially in the Mediterranean basin, fires are mostly human-caused mainly due arson. The distance to transport networks and the distance to urban or recreation areas are among the most frequently used human factors in modelling exercises and the Wildland-Urban Interface is increasingly taken into account in the modelling of fire occurrence. Depending on the socio-economic context of the region concerned, factors such as the unemployment rate or variables linked to agricultural activity can explain the ignition of intentional and unintentional fires. Regarding environmental factors, those related to weather, fuel and topography are the most significant drivers of ignition of forest fires, especially in Mediterranean-type regions. For both human and lightning-caused fires, there is a geographical gradient of fire ignition, mainly due to variations in climate and fuel composition but also to population density for instance. The timing of fires depends on their causes. In populated areas, the timing of human-caused fires is closely linked to human activities and peaks in the afternoon whereas, in remote areas, the timing of lightning-caused fires is more linked to weather conditions and the season, with most such fires occurring in summer. 相似文献
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SUMMARY A participatory research programme was conducted in the groundnut basin of Senegal, where there was a need to intensify the local farming systems. The research yielded a set of technical references and profitable innovations, which were introduced through specific development actions. The technology transfer aims at the establishment of sustainable farming systems and takes into account the climatic risks and innovation costs. Identification of research themes and implementation of results was based on various analyses: climatic analysis at regional level (establishment of climatic zones) and identification of constraints at farm level. Farmers' interests and the research results were combined to define suitable interventions for each surveyed farm, with the aim of increasing farm income and restoring soil fertility. The research also yielded elements for formulating an appropriate and realistic agricultural policy for the region. Clearly, the development of livestock farming activities is essential in bringing new economic life into the region and increasing the sustainability of farming systems in erratic climatic conditions. 相似文献
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