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1.
Fetal aspartylglucosaminuria (AGU) was studied during the first trimester of pregnancy in six at-risk pregnancies using chorionic villus samples. The activity of aspartylglucosaminidase (AGA) was high in five cases, indicating an unaffected fetus. This was confirmed through delivery of healthy newborns with a normal pattern of urinary oligosaccharides. Low enzyme activity in an uncultured biopsy specimen and in cultured amniotic fluid cells in one case demonstrated that the fetus was affected. The pregnancy was terminated and the prenatal diagnosis was confirmed by showing reduced AGA activity in cultured fibroblasts of the fetus.  相似文献   
2.
Kuuluvainen T  Tahvonen O  Aakala T 《Ambio》2012,41(7):720-737
Since WWII, forest management in Fennoscandia has primarily been based on even-aged stand management, clear cut harvesting and thinning from below. As an alternative, uneven-aged management, based on selection cutting of individual trees or small groups of trees, has been proposed. In this review we discuss the theoretical aspects of ecology and economics of the two management approaches. We also review peer-reviewed studies from boreal Fennoscandia, which have aimed at comparing the outcomes of uneven-aged and the conventional even-aged forest management. According to a common view the main obstacle of practicing uneven-aged forestry is its low economic performance. However, the reviewed studies did not offer any straightforward support for this view and several studies have found uneven-aged management to be fully competitive with existing even-aged management. Studies on the ecological aspects indicated that selection cuttings maintain mature or late-successional forest characteristics and species assemblages better than even-aged management, at least at the stand scale and in the short term. We conclude that although the number of relevant studies has increased in recent years, the ecological and economic performance of alternative management methods still remains poorly examined, especially for those stands with multiple tree species and also at wider spatial and temporal scales. For future research we advocate a strategy that fully takes into consideration the interdisciplinary nature of forest management and is better connected to social goals and latest theoretical and methodological developments in ecology and economics.  相似文献   
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The structure of the habitat is usually crucial for growth and survival of young life stages. Presently, some nursery areas of fish larvae are changing due to eutrophication, e.g. due to enhanced growth of ephemeral filamentous algae at the expense of perennial species. We studied the influence of two habitats, one with filamentous algae (Cladophora glomerata) and the other with bladder wrack (Fucus vesiculosus), on habitat choice of pike larvae (Esox lucius) in the absence/presence of a predator or a competitor. We further tested whether the habitat choice is adaptive in increasing survival under predation threat. In contrast to expectations, pike larvae preferred the habitat with ephemeral filamentous algae to the bladder wrack, thriving in clean waters, independent of the presence/absence of both predator/competitor. In addition, the survival of the larvae was higher in the filamentous algae in the presence of predators, which suggested that the habitat preference of the larvae was adaptive. The structure of the bladderwrack habitat was probably too open for newly hatched larvae, which implies that F. vesiculosus and other large brown algae are not as important refuges for young larvae as previously thought.  相似文献   
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ABSTRACT

Small and medium-sized enterprises (SMEs) have a substantial role in the economy and job creation, but they are a remarkable source of environmental impacts. SMEs often lack skills and resources to compile environmental impact assessments; Streamlined Life Cycle Analysis (LCA) can provide efficient tools for this. An application of streamlined LCA relying heavily on database data, LCA clinic, was developed and tested on 23 SMEs in Finland. The climate change impacts were mainly caused by the production of raw materials, electricity and heating, whereas packaging and transportation were not influential. A significant amount of emissions were indirect, i.e. caused by production of raw materials. Thus, decreasing emissions from raw material production or selecting raw materials with a smaller environmental load could be a more efficient way to decrease emissions than reducing direct emissions such as those from electricity use. Lack of data in the LCA-databases was considered a challenge. An access to regionally customised datasets is important for the implementation of LCA clinics. Company feedback indicated that LCA clinics were useful in climate-friendly product design and increased environmental awareness, but did not lead to immediate actions to reduce emissions because of inadequate investment capabilities. Company managers had limited possibilities to use the results in marketing as comparative assessments would require a full LCA. Many company managers were willing to pay a fee sufficient to cover the costs of an LCA clinic, but some considered that the costs should be covered by external funding sources.  相似文献   
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Which organisations saw their positions on two contentious issues reflected in the Irish climate law of 2015, and what role did advocacy coalitions play in the policy process? These questions are answered drawing theoretically from the Advocacy Coalition Framework (ACF) and by conducting a network analysis of survey data collected from the organisations involved in the national climate policy process. The study finds that several institutionally important or economically powerful organisations, particularly those involved in the agricultural sector, as well as the government parties saw their preferences reflected in the law. This resulted in legislation that excluded binding emission reductions targets, differentiating it from similar laws introduced in other European countries. Organisations in favour of stronger regulation formed a coalition to advocate for their positions, but they largely failed to get their way.  相似文献   
8.
An environmentally extended input-output (EE-IO) analysis - environmental impacts of material flows caused by the Finnish economy - was carried out in order to improve data on production and consumption in Finland. The study resulted in the ENVIMAT model, which can be used to analyze the relationship between material flows, environmental impacts and the economy. The model is based on monetary and physical input-output tables and an environmental life-cycle impact assessment. This article summarizes the main methodological aspects and findings regarding the material flows and climate impacts caused by the Finnish economy in 2002 and 2005. The Finnish model has relatively detailed input data with 150 industries and 918 products and the data on imports was assessed according to a mixed approach with the help of life-cycle inventory data. The results of the model showed that the Finnish economy uses imported material resources as much as domestic resources. Life-cycle greenhouse gas (GHG) emissions caused by imports were equivalent to 70-80% of domestic emissions. The GHG emissions embodied in imports (emissions abroad) and exports (emissions within Finland) were of the same magnitude. The analysis showed that the service sector accounted for 44% of GHG emissions caused by the domestic final use of products. Analysis of the results also showed that the indicator of total material requirement (TMR) should not be used for environmental impact comparisons of products and services. In the future, the aim is to use the ENVIMAT model for assessing temporal changes in the economy; for monitoring sustainable development; for planning climate change mitigation; and for identifying important factors in the economy and assessing their impacts.  相似文献   
9.
Honeybees present a paradox that is unusual among the social Hymenoptera: extremely promiscuous queens generate colonies of nonreproducing workers who cooperate to rear reproductives with whom they share limited kinship. Extreme polyandry, which lowers relatedness but creates within-colony genetic diversity, produces substantial fitness benefits for honeybee queens and their colonies because of increased disease resistance and workforce productivity. However, the way that these increases are generated by individuals in genetically diverse colonies remains a mystery. We assayed the foraging and dancing performances of workers in multiple-patriline and single-patriline colonies to discover how within-colony genetic diversity, conferred to colonies by polyandrous queens, gives rise to a more productive foraging effort. We also determined whether the initiation by foragers of waggle-dance signaling in response to an increasing sucrose stimulus (their dance response thresholds) was linked to patriline membership. Per capita, foragers in multiple-patriline colonies visited a food source more often and advertised it with more waggle-dance signals than foragers from single-patriline colonies, although there was variability among multiple-patriline colonies in the strength of this difference. High-participation patrilines emerged within multiple-patriline colonies, but their more numerous foragers and dancers were neither more active per capita nor lower-threshold dancers than their counterparts from low-participation patrilines. Our results demonstrate that extreme polyandry does not enhance recruitment effort through the introduction of low-dance-threshold, high-activity workers into a colony’s population. Rather, genetic diversity is critical for injecting into a colony’s workforce social facilitators who are more likely to become engaged in foraging-related activities, so boosting the production of dance signals and a colony’s responsiveness to profitable food sources.  相似文献   
10.
The ecological impacts of extreme climatic events on population dynamics and community composition are profound and predominantly negative. Using extensive data of an ecological model system, we tested whether predictions from ecological models remain robust when environmental conditions are outside the bounds of observation. We observed a 10-fold demographic decline of the Glanville fritillary butterfly (Melitaea cinxia) metapopulation on the Åland islands, Finland in the summer of 2018 and used climatic and satellite data to demonstrate that this year was an anomaly with low climatic water balance values and low vegetation productivity indices across Åland. Population growth rates were strongly associated with spatiotemporal variation in climatic water balance. Covariates shown previously to affect the extinction probability of local populations in this metapopulation were less informative when populations were exposed to severe drought during the summer months. Our results highlight the unpredictable responses of natural populations to extreme climatic events.  相似文献   
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