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681.
Mitigation and Adaptation Strategies for Global Change - Bioenergy with carbon dioxide (CO2) capture and storage (BECCS) technologies represent an interesting option to reach negative carbon...  相似文献   
682.
Tuberous sclerosis (TSC) is a frequent autosomal-dominant condition (affecting 1 in 6000 individuals) caused by various mutations in either the hamartin (TSC1) or the tuberin gene (TSC2). This allelic and non-allelic heterogeneity makes genetic counseling and prenatal diagnosis difficult, especially as a significant proportion of TSC cases are due to de novo mutations. For this reason the identification of the disease causing mutation is mandatory for accurate counseling, yet current mutation detection methods such as single-strand conformation polymorphism (SSCP) or denaturing gradient gel electrophoresis (DGGE) are labor intensive with limited detection efficiency. Denaturing high-performance liquid chromatography (DHPLC) is a high-throughput, semi-automated mutation detection system with a reported mutation detection rate close to 100% for PCR fragments of up to 800 bp. We used a recently described DHPLC assay allowing the efficient detection of mutations in TSC1 to analyze the DNA extracted from a chorion villus sample in order to perform a prenatal diagnosis for TSC. The fetus was found not to have inherited the deleterious mutation and the DHPLC diagnosis was confirmed by haplotype analysis. This represents the first DHPLC-based prenatal diagnosis of a genetic disease. Copyright © 2001 John Wiley & Sons, Ltd.  相似文献   
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The anti-predator behaviour of Baltic crustacean planktivores was studied in feeding experiments under predation pressure of herring. The experiments were conducted with pelagic mysids: Mysis mixta and Mysis relicta, and with Cercopagis pengoi, a non-indigenous cladoceran, which invaded the Baltic Sea in 1992. Zooplankton was offered as prey. Two kinds of experiments were performed in the absence and presence of chemical predator cues: (1) two-prey experiments with prey, which have poor or good escape responses and all three planktivores and (2) natural prey experiments with mysids in natural zooplankton assemblages. The results showed that all three species reacted to the chemical cue of herring by decreasing their feeding rate and altering prey selection. C. pengoi selected easily captured prey (rotifers) in two-prey experiments under predation risk while selection for any prey was evident in mysids in natural prey experiments only in the absence of predator cues. This indicates that planktivores have different anti-predator strategies, which are modified by their own prey capture abilities. C. pengoi was a very efficient predator on small prey with size-specific prey consumption rate 5 to 18 times the rate of mysids. Results show that the studied planktivores are capable of adjusting their feeding behaviour to decrease their conspicuousness in order to increase survival under predation risk. Further, results support the view that C. pengoi has adapted well to the Baltic ecosystem, sharing food niche with pelagic mysids and most probably having a strong influence on the whole pelagic food web.  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   
687.
When population is increasing, characterizing the optimal water consumption path is complicated by the fact that the underlying dynamics of the water stock is contingent on the level of the stock itself. We propose a method of constructing the optimal path in this case. Since population is increasing, the optimal consumption path may involve refraining at times from consuming the totality of the surface water flow in order to restock in groundwater for future consumption. The aquifer then serves as a means to achieve welfare increasing intertemporal transfers of surface water. Therefore the aquifer itself, as distinct from the stock of water it serves to store, may have value and the marginal valuation of water when groundwater stocks are being drawn upon should, for this reason, differ at times from the marginal valuation of water when it is drawn strictly from surface water.  相似文献   
688.
The objective of this work was to compare the performance of two laboratory-scale, mesophilic systems aiming at the anaerobic digestion of the organic fraction of municipal solid wastes (OFMSW). The first system consisted of two coupled reactors packed with OFMSW (PBR1.1-PBR1.2) and the second system consisted of an upflow anaerobic sludge bed reactor (UASB) coupled to a packed reactor (UASB2.1-PBR2.2). For the start-up phase, both reactors PBR 1.1 and the UASB 2.1 (also called leading reactors) were inoculated with a mixture of non-anaerobic inocula and worked with leachate and effluent full recirculation, respectively. Once a full methanogenic regime was achieved in the leading reactors, their effluents were fed to the fresh-packed reactors PBR1.2 and PBR2.2, respectively. The leading PBR 1.1 reached its full methanogenic regime after 118 days (Tm, time to achieve methanogenesis) whereas the other leading UASB 2.1 reactor reached its full methanogenesis regime after only 34 days. After coupling the leading reactors to the corresponding packed reactors, it was found that both coupled anaerobic systems showed similar performances regarding the degradation of the OFMSW. Removal efficiencies of volatile solids and cellulose and the methane pseudo-yield were 85.95%, 80.88% and 0.109 NL CH4 g(-1) VS(fed) in the PBR-PBR system; and 88.75%, 82.61% and 0.115 NL CH4 g(-1) VS(fed0 in the UASB-PBR system [NL, normalized litre (273 degrees K, 1 ata basis)]. Yet, the second system UASB-PBR system showed a faster overall start-up.  相似文献   
689.
The examinations conducted in this study focus at the community level to test for disparate outcomes involving utility-based electric power generation within the crucial state of Texas. Potential policy implications are discussed as relevant to the general thesis of environmental racism postulated by justice advocates and the civil rights strategies advanced by justice advocates. Cross-sectional and longitudinal perspectives are used in testing for distributive inequities involving locations of fossil fuel power plants and emissions and output rates of emissions originating from such power plants. These tests provide empirical basis for assessing outcomes of the siting and emissions regulatory processes. While the civil rights strategies that would use Title VI of the Civil Rights Act of 1964 and the disparate impact standard are inapplicable, some limited findings indicate disparate outcomes involving other disadvantaged populations that are difficult to justify in context of legitimate market dynamics. Issues raised in this study have relevance to national energy policy proposals that promote many more power plants across the USA and encourage emissions trading.  相似文献   
690.
The main difficulty in developing a molecular diagnosis of spinal muscular atrophy (SMA) resides in the specific genomic structure of the locus. Indeed, two highly homologous survival motor neurone genes, SMN1 and SMN2, are present at the locus. The detection of the homozygous deletion of exons 7 and 8 of the SMN1 gene, which is present in 90 to 98% of the patients, is based on methods highlighting 1 of the 8 nucleotidic mismatches existing between these 2 genes. In order to offer preimplantation genetic diagnosis (PGD) for SMA, we developed a new allele-specific amplification method. The main disadvantage of our previously described strategy resided in the possibility of diagnosing, in case of amplification failure, an unaffected embryo as affected. We present here a new PGD-SMA method. We established the conditions for three different duplex PCRs, allowing the specific detection of the SMN1 gene and one polymorphic marker, either D5S629, D5S1977, or D5S641. Of the 60 to 90 single cells tested, the PCR efficiency varied from 98 to 100% with a complete genotype obtained in a range between 81 and 87% with a global allele drop-out rate of 9%. Such a test was used to perform 1 PGD cycle for which 7 embryos could be analysed. All the embryos were fully diagnosed, six as unaffected and one as affected. Four embryos were transferred, but no pregnancy ensued. Copyright © 2003 John Wiley & Sons, Ltd.  相似文献   
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