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Amniocentesis and subsequent tests are reported on a fetus conceived of a rare mating type: its mother has an intermediate level of β hexosaminidase A (HEX A), characteristic of carriers of Tay-Sachs disease (TSD), while the father suffers from an adult-onset GM2 ganglio-sidosis (AOG) with severe HEX A deficiency. Activity of HEX A in the cultured fetal cells was very low when measured by the heat-inactivation method, thus showing the typical biochemical phenotype of TSD fetuses. However, upon separation of HEX isozymes by ion exchange chromatography, residual HEX A (17 per cent of total HEX) was demonstrated. Also in contrast to TSD fetuses, this fetus' fibroblasts were able to synthesize the precursor of a chains of HEX, and ultrastructural examination of its brain revealed few atypical lamellar bodies, unlike those found in TSD fetuses of the same gestational age. It is therefore concluded that the fetus was not affected with TSD, but rather with AOG. 相似文献
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Maria J. Restrepo Margareta A. Lelea Brigitte A. Kaufmann 《Sustainability Science》2018,13(5):1265-1286
Although knowledge integration and co-production are integral to transdisciplinary approaches to foster sustainable change in social–ecological systems, this type of research is usually not evaluated based on assessments of the learning process. While participants are meant to be central in such approaches, too often, their perspectives are not central to the evaluation. Moreover, there is limited empirical information about how new knowledge is transformed into action. We respond to these knowledge gaps by analyzing (A) farmers’ perspectives on the collaborative learning process and (B) how farmers’ new knowledge can serve as the basis for changed actions. Theoretically, we are guided by second-order cybernetics and have integrated the Control Loop Model with Learning Loops to extend Kirkpatrick (Evaluating training programs: the four levels, 2nd edn. Berrett-Koehler Publisher, San Francisco, 1998) four-level evaluation scheme. We apply this to evaluate a 2-year collaborative learning process with two smallholder dairy farmer groups in Nakuru County, Kenya that aimed to co-develop local sustainable pathways to reduce milk losses. Results showed that farmers learned by (1) implementing corrective actions based on known cause–effect relations (single-loop learning); (2) discovering new cause–effect relations and testing their effect (double-loop learning); and (3) further questioning and changing their aims (triple-loop learning). Highlighting the importance of knowledge integration and co-production, this collaboration between farmers, researchers, and field assistants improved the farmers’ ability to respond, adapt, and intentionally transform their farming system in relation with complex sustainability challenges. Results demonstrate that the potential of our evaluation scheme to better reflect learning and empowerment experienced by actors involved in transdisciplinary research for sustainability. 相似文献
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Joshua Eli Cinner Morgan Stuart Pratchett Nicholas Anthony James Graham Vanessa Messmer Mariana Menezes Prata Bezerra Fuentes Tracy Ainsworth Natalie Ban Line Kolind Bay Jessica Blythe Delphine Dissard Simon Dunn Louisa Evans Michael Fabinyi Pedro Fidelman Joana Figueiredo Ashley John Frisch Christopher John Fulton Christina Chemtai Hicks Vimoksalehi Lukoschek Jennie Mallela Aurelie Moya Lucie Penin Jodie Lynn Rummer Stefan Walker David Hall Williamson 《Regional Environmental Change》2016,16(4):1133-1146
Corals and coral-associated species are highly vulnerable to the emerging effects of global climate change. The widespread degradation of coral reefs, which will be accelerated by climate change, jeopardizes the goods and services that tropical nations derive from reef ecosystems. However, climate change impacts to reef social–ecological systems can also be bi-directional. For example, some climate impacts, such as storms and sea level rise, can directly impact societies, with repercussions for how they interact with the environment. This study identifies the multiple impact pathways within coral reef social–ecological systems arising from four key climatic drivers: increased sea surface temperature, severe tropical storms, sea level rise and ocean acidification. We develop a novel framework for investigating climate change impacts in social–ecological systems, which helps to highlight the diverse impacts that must be considered in order to develop a more complete understanding of the impacts of climate change, as well as developing appropriate management actions to mitigate climate change impacts on coral reef and people. 相似文献
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Burkhard Schmidt Jürgen Ebert MARC Lamshöft Brigitte Thiede Ramona Schumacher-Buffel Rong Ji 《Journal of environmental science and health. Part. B》2013,48(1):8-20
The fate of 14C-labeled sulfadiazine (14C-SDZ) residues was studied in time-course experiments for 218 days of incubation using two soils (Ap horizon of loamy sand, orthic luvisol; Ap horizon of silt loam, cambisol) amended with fresh and aged (6 months) 14C-manure [40 g kg?1 of soil; 6.36 mg of sulfadiazine (SDZ) equivalents per kg of soil], which was derived from two shoats treated with 14C-SDZ. Mineralization of 14C-SDZ residues was below 2% after 218 days depending little on soil type. Portions of extractable 14C (ethanol-water, 9:1, v/v) decreased with time to 4–13% after 218 days of incubation with fresh and aged 14C-manure and both soils. Non-extractable residues were the main route of the fate of the 14C-SDZ residues (above 90% of total recovered 14C after 218 days). These residues were high immediately after amendment depending on soil type and aging of the 14C-manure, and were stable and not remobilized throughout 218 days of incubation. Bioavailable portions (extraction using CaCl2 solution) also decreased with increasing incubation period (5–7% after 218 days). Due to thin-layer chromatography (TLC), 500 μg of 14C-SDZ per kg soil were found in the ethanol-water extracts immediately after amendment with fresh 14C-manure, and about 50 μg kg?1 after 218 days. Bioavailable 14C-SDZ portions present in the CaCl2 extracts were about 350 μg kg?1 with amendment. Higher concentrations were initially detected with aged 14C-manure (ethanol-water extracts: 1,920 μg kg?1; CaCl2 extracts: 1,020 μg kg?1), probably due to release of 14C-SDZ from bound forms during storage. Consistent results were obtained by extraction of the 14C-manure-soil samples with ethyl acetate; portions of N-acetylated SDZ were additionally determined. All soluble 14C-SDZ residues contained in 14C-manure contributed to the formation of non-extractable residues; a tendency for persistence or accumulation was not observed. SDZ's non-extractable soil residues were associated with the soluble HCl, fulvic acids and humic acids fractions, and the insoluble humin fraction. The majority of the non-extractable residues appeared to be due to stable covalent binding to soil organic matter. 相似文献
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Predictive value of cardiovascular parameters in stages 1 and 2 of twin-to-twin transfusion syndrome
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Rachèl V. van Schendel G. C. Page-Christiaens Lean Beulen Catia M. Bilardo Marjon A. de Boer Audrey B. C. Coumans Brigitte H. Faas Irene M. van Langen Klaske D. Lichtenbelt Merel C. van Maarle Merryn V. E. Macville Dick Oepkes Eva Pajkrt Lidewij Henneman for the Dutch NIPT Consortium 《黑龙江环境通报》2016,36(12):1091-1098