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The de-O-sulphation of α-linked glucosamine-6-sulphate residues in heparan sulphate requires a specific sulphatase, glucosamine-6-sulphatase, which has been shown to be deficient in tissues of Sanfilippo D, or mucopolysaccharidosis type IIID (MPS IIID), patients. MPS IIID fibroblasts cultured in Basal Eagle's medium supplemented with either fetal calf serum or heat-inactivated fetal calf serum, MDCB or Ultraserg media had residual glucosarnine-6-sulphatase activities towards a heparin-derived trisaccharide substrate, O-(α-N-acetylglucosamine-6-sulphate)-(1→4)-L -O-(α-iduronic acid-2-sulphate)-(1→4)-D -O-2,5-anhydro [1-3H]mannitol-6-sulphate, GlcNAc6S-IdoA2S-anM6S, which were less than 1 per cent of the normal range for fibroblasts cultured in Basal Eagle's medium supplemented with fetal calf serum. However, the glucosamine-6-sulphatase activities of MPS IIID fibroblasts grown in Chang's medium were similar to the activities in normal control fibroblasts which were cultured in Basal Eagle's medium. These results indicate that caution is required for prenatal diagnosis of MPS IIID patients using chorionic villi or amniotic cells cultured in Chang's medium. 相似文献
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Scholz C. Jones T. G. West M. Ehbair A. M. S. Dunn C. Freeman C. 《Environmental science and pollution research international》2016,23(18):18192-18199
Environmental Science and Pollution Research - The objective of this study was to monitor a newly constructed wetland (CW) in north Wales, UK, to assess whether it contributes to an improvement in... 相似文献
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van Heezik Yolanda Freeman Claire Davidson Katherine Lewis Blake 《Environmental management》2020,66(1):42-55
Environmental Management - Activities undertaken by householders in their gardens have huge potential to enhance city-wide biodiversity, but programs aimed at activating householders require an... 相似文献
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BRENT J. SEWALL AMY L. FREESTONE MOHAMED F. E. MOUTUI NASSURI TOILIBOU ISHAKA SAÏD SAINDOU M. TOUMANI DAOUD ATTOUMANE CHEIKH M. IBOURA 《Conservation biology》2011,25(4):688-696
Abstract: Systematic conservation assessment (an information‐gathering and prioritization process used to select the spatial foci of conservation initiatives) is often considered vital to conservation‐planning efforts, yet published assessments have rarely resulted in conservation action. Conservation assessments may lead more directly to effective conservation action if they are reoriented to inform conservation decisions. Toward this goal, we evaluated the relative priority for conservation of 7 sites proposed for the first forest reserves in the Union of the Comoros, an area with high levels of endemism and rapidly changing land uses in the western Indian Ocean. Through the analysis of 30 indicator variables measured at forest sites and nearby villages, we assessed 3 prioritization criteria at each site: conservation value, threat to loss of biological diversity from human activity, and feasibility of reserve establishment. Our results indicated 2 sites, Yiméré and Hassera‐Ndrengé, were priorities for conservation action. Our approach also informed the development of an implementation strategy and enabled an evaluation of previously unexplored relations among prioritization criteria. Our experience suggests that steps taken to ensure the closer involvement of practitioners, include a broader range of social data, encourage stakeholder participation, and consider the feasibility of conservation action can improve the relevance of assessments for conservation planning, strengthen the scientific basis for conservation decisions, and result in a more realistic evaluation of conservation alternatives. 相似文献
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Globally, the issue of energy use and climate change is at the forefront of many national and international agendas, and also an issue central to children's lives and well-being. This paper examines how children are both affected by and contribute to the problems associated with unsustainable energy use as it relates to the growth of car dependent lives. The paper presents the findings of a study of travel as it relates to the everyday lives of children in the city of Dunedin in New Zealand. The study found that children currently lead complex car dependent lives. This extensive car reliance presents a serious and largely unrecognised challenge to the sustainable planning and management of the urban environment. The move towards more sustainable transportation can bring benefits for both the environment and for children's lives. 相似文献
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N.C.G. Freeman T. Wainman P.J. Lioy A.H. Stern S.I. Shupack 《Journal of the Air & Waste Management Association (1995)》2013,63(8):604-605
Honorary Memberships are conferred by the Air Pollution Control Association on persons of widely recognized eminence in some part of the field of air pollution control or who have rendered especially meritorious service to the Association. Honorary Memberships were awarded this year to Dr. Leonard Greenburg and Mr. Milton Reizenstein, both past presidents of APCA. 相似文献