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We report an interstitial deletion of chromosome 3q26-q28 in a fetus in which anophthalmia had been detected prenatally. FISH analysis, using BAC clones encompassing the SOX2 locus, showed that SOX2 gene was involved in the chromosomal breakpoint of the deletion. This case confirms that haploinsufficiency for SOX2 plays a crucial role in human eye development and emphasizes the necessity of careful chromosomal analysis, including FISH analysis of the 3q region, in case of prenatal discovery of anophthalmia. Copyright © 2004 John Wiley & Sons, Ltd.  相似文献   
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Two male siblings with several malformations are reported. The anomalies detected in both fetuses were mesomelic camptomelia, postaxial hexadactyly and Dandy–Walker complex. There was only one similar previous report in the literature. This combination could represent a specific pattern of malformation or a new syndrome, with different variants. The parents' consanguinity and the recurrence in a subsequent pregnancy suggest an autosomal recessive inheritance pattern. Copyright © 2003 John Wiley & Sons, Ltd.  相似文献   
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In line with the resource curse literature, this paper examines the effect of oil dependency on the disparities in access to electricity between urban and rural areas in Africa, conditional on the quality of political institutions. Based on data from 36 African countries over the period 2000–2017, our investigation suggests that oil rents (% of GDP) increase urban–rural disparities in access to electricity. However, the quality of institutions shapes the effect of oil dependency on these disparities. Specifically, a 10% increase in the institutional quality score reduces the adverse effects of oil rent on electricity access disparity by around 19%, and the negative impact of oil dependency on urban–rural disparities is reversed when institutional quality reaches a score of 52% on a scale from 0 to 100. The robustness tests support these results and call for strengthening the quality of institutions to overcome the resource curse in Africa.  相似文献   
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The hazardous effect of dynamic pressure and strong gas flows induced by a methane–air mixture explosion in underground coal mines is studied. The dynamic pressure effect of a methane–air explosion was analyzed by numerical simulation, in a duct and tunnel. Compared to the overpressure generated by an explosion that can act on a body, the dynamic pressure caused by the high-speed flow of the gaseous combustion products can cause serious damage as well. At the structural opening of a coal mine, the destruction caused by the dynamic pressure induced by a methane–air explosion is more serious than the overpressure. For a tube or tunnel partially filled by a methane–air mixture, the dynamic pressure is lower than the overpressure in the region occupied by the flammable mixture. Beyond the premixed region, the dynamic pressure is of the same order of magnitude as the overpressure.  相似文献   
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Former ranges of wild animals have been reestablished in many developed countries. However, this reestablishment has led to increasing human–wildlife conflict in agroforest ecosystems. In Japan, human–wildlife conflict, such as crop raiding by and ecological impacts of wild ungulates and primates, is a serious problem in depopulated rural areas due to these animal range expansions and increased abundances. Japan's human population is predicted to decline by 24% by 2050, and approximately 20% of agricultural settlements will become completely depopulated. In this scenario, anthropogenic pressures on wildlife (e.g., hunting and habitat alteration) will continue to decrease and human–wildlife conflict will increase due to increasing wildlife recovery. Japan's local governments plan to slow range recovery, prevent species reestablishment, or remove recolonizing large mammals through lethal control. This strategy, however, is not cost-effective, and workforce shortages in depopulated communities make it infeasible. Moreover, the suppression of wildlife prevents the recovery of ecological functions and thus would degrade regional biodiversity. The declining pressure on wildlife that accompanies human depopulation will prevent the restoration of any past states of human–wildlife interaction. We suggest human-used areas in rural landscapes be aggregated in compact cities and that in transition zones between human settlements and depopulated lands that land-sharing approaches be applied. Concentrating management efforts in compact cities may effectively decrease human–wildlife conflict, rather than intensifying human pressures. Reforestation of depopulated lands may lead to recovery of wildlife habitats, their ecosystem functions, and regional biodiversity due to minimization of negative anthropogenic effects (land-sparing approach). Balancing resolution of human–wildlife conflict and ecological rewilding could become a new, challenging task for regional wildlife managers.  相似文献   
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An attempt has been made to produce stable water–diesel emulsion with optimal formulation and process parameters and to evaluate the performance and emission characteristics of diesel engine using this stable water–diesel emulsion. A total of 54 samples were prepared with varying water/diesel ratio, surfactant amount and stirring speed and water separation was recorded after 24 and 48 hr of emulsification. The recorded data were used in artificial neural network (ANN)-particle swarm optimization (PSO) technique to find the optimal parameters to produce water–diesel emulsion for engine testing. The predicted optimal parameters were found as 20% water to diesel ratio, 0.9% surfactant and 2200 rpm of stirrer for a water separation of 14.33% in one day with a variation of 6.54% against the actual value of water separation. Water–diesel emulsion fuel exhibited similar fuel properties as base fuel. The peak cylinder gas pressure, peak pressure rise rate and peak heat release rate for water–diesel were found higher as compared to diesel at medium to full engine loads. The improved air-fuel mixing in water–diesel emulsion enhanced brake thermal efficiency (BTE) of engine. The absorption of heat by water droplets present in water–diesel emulsion led to reduced exhaust gas temperature (EGT). With water–diesel emulsion fuel, the mean carbon monoxide (CO), unburned hydrocarbon and oxides of nitrogen (NOx) emissions reduced by 8.80, 39.60, and 26.11%, respectively as compared to diesel.  相似文献   
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Wildlife conservation and management (WCM) practices have been historically drawn from a wide variety of academic fields, yet practitioners have been slow to engage with emerging conversations about animals as complex beings, whose individuality and sociality influence their relationships with humans. We propose an explicit acknowledgement of wild, nonhuman animals as active participants in WCM. We examined 190 studies of WCM interventions and outcomes to highlight 3 common assumptions that underpin many present approaches to WCM: animal behaviors are rigid and homogeneous; wildlife exhibit idealized wild behavior and prefer pristine habitats; and human–wildlife relationships are of marginal or secondary importance relative to nonhuman interactions. We found that these management interventions insufficiently considered animal learning, decision-making, individuality, sociality, and relationships with humans and led to unanticipated detrimental outcomes. To address these shortcomings, we synthesized theoretical advances in animal behavioral sciences, animal geographies, and animal legal theory that may help conservation professionals reconceptualize animals and their relationships with humans. Based on advances in these fields, we constructed the concept of animal agency, which we define as the ability of animals to actively influence conservation and management outcomes through their adaptive, context-specific, and complex behaviors that are predicated on their sentience, individuality, lived experiences, cognition, sociality, and cultures in ways that shape and reshape shared human–wildlife cultures, spaces, and histories. Conservation practices, such as compassionate conservation, convivial conservation, and ecological justice, incorporate facets of animal agency. Animal agency can be incorporated in conservation problem-solving by assessing the ways in which agency contributes to species’ survival and by encouraging more adaptive and collaborative decision-making among human and nonhuman stakeholders.  相似文献   
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