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Prenatal diagnosis of Sandhoff disease (infantile onset) at 16 weeks gestation has been made by detection and analysis of N-acetylglucosaminyl-oligosaccharides in amniotic fluid using high performance liquid chromatography. The elution profile for the branched chain oli-gosaccharides was identical with that obtained with neonatal and infantile Sandhoff urine. The concentration of the oligosaccharides in the fluid was 1/100th that of urine but when calculated relative to creatinine the levels were similar. No oligosaccharides were detected in normal control amniotic fluids (10 patients) at a similar gestational age. Based on the levels of the amniotic fluid oligosaccharides and the sensitivity limits of the assay, prenatal diagnosis of patients with the juvenile onset form of the disease may also be possible with this technique. 相似文献
704.
At 10 weeks' gestation, chorionic villus biopsy was obtained from a woman at risk for Sanfilippo type C disease. Acetyl-CoA: α-glucosaminide N-acetyltransferase activity was markedly deficient. The pregnancy was terminated at 12 weeks' gestation and follow-up study on fetal fibroblasts confirmed the diagnosis. 相似文献
705.
Bilateral cleft lip and cleft palate can be diagnosed by ultrasonography prior to 20 weeks of pregnancy. The anomaly produces an abnormal facial profile and, on cross-section, the clefts in the maxilla are demonstrable. The method is illustrated by sonograms from a fetus in which the defect was diagnosed before trisomy 13 became known by karyotyping. 相似文献
706.
High-performance permanent magnets 总被引:2,自引:0,他引:2
High-performance permanent magnets (pms) are based on compounds with outstanding intrinsic magnetic properties as well as
on optimized microstructures and alloy compositions. The most powerful pm materials at present are RE–TM intermetallic alloys
which derive their exceptional magnetic properties from the favourable combination of rare earth metals (RE=Nd, Pr, Sm) with
transition metals (TM=Fe, Co), in particular magnets based on (Nd,Pr)2Fe14B and Sm2(Co,Cu,Fe,Zr)17. Their development during the last 20 years has involved a dramatic improvement in their performance by a factor of >15 compared
with conventional ferrite pms therefore contributing positively to the ever-increasing demand for pms in many (including new)
application fields, to the extent that RE–TM pms now account for nearly half of the worldwide market. This review article
first gives a brief introduction to the basics of ferromagnetism to confer an insight into the variety of (permanent) magnets,
their manufacture and application fields. We then examine the rather complex relationship between the microstructure and the
magnetic properties for the two highest-performance and most promising pm materials mentioned. By using numerical micromagnetic
simulations on the basis of the Finite Element technique the correlation can be quantitatively predicted, thus providing a
powerful tool for the further development of optimized high-performance pms. 相似文献
707.
H. Kühne 《Die Naturwissenschaften》1957,44(15):431-431
708.
709.
William G. Coleman 《Environmental management》1996,20(6):815-825
The term biodiversity describes the array of interacting, genetically distinct populations and species in a region, the communities they comprise, and the variety of ecosystems of which they are functioning parts. Ecosystem health, a closely related concept, is described in terms of a process identifying biological indicators, end points, and values. The decline of populations or species, an accelerating trend worldwide, can lead to simplification of ecosystem processes, thus threatening the stability and sustainability of ecosystem services directly relevant to human welfare in the chain of economic and ecological relationships. The challenge of addressing issues of such enormous scope and complexity has highlighted the limitations of ecology-as-science. Additionally, biosphere-scale conflicts seem to lie beyond the scope of conventional economics, leading to differences of opinion about the commodity value of biodiversity and of the services that intact ecosystems provide. In the face of these uncertainties, many scientists and economists have adopted principles that clearly assign burdens of proof to those who would promote the loss of biodiversity and that also establish near-trump (preeminent) status for ecological integrity. Electric utility facilities and operations impact biodiversity whenever construction, operation, or maintenance of generation, delivery, and support facilities alters landscapes and habitats and thereby impacts species. Although industry is accustomed to dealing with broad environmental concerns (such as global warming or acid rain), the biodiversity issue invokes hemisphere-wide, regional, local, and site-specific concerns all at the same time. Industry can proactively address these issues of scope and scale in two main ways: first, by aligning strategically with the broad research agenda put forth by informed scientists and institutions; and second, by supporting focused management processes whose results will contribute incrementally to the broader agenda of rebuilding or maintaining biodiversity. 相似文献
710.
Model oligo esters of terephthalic acid with 1,2-ethanediol, 1,3-propanediol, and 1,4-butanediol have been investigated with regard to their biodegradability in different biological environments. Well-characterized oligomers with weight-average molar masses of from 600 to 2600 g/mol exhibit biodegradation in aqueous systems, soil, and compost at 60°C. SEC investigations showed a fast biological degradation of the oligomer fraction consisting of 1 or 2 repeating units, independent of the diol component used for polycondensation, while polyester oligomers with degrees of polymerization higher than 2 were stable against microbial attack at room temperature in a time frame of 2 months. At 60°C in a compost environment chemical hydrolysis also degrades chains longer than two repeating units, resulting in enhanced degradability of the oligomers. Metabolization of the monomers and the dimers as well by the microorganisms could be confirmed by comparing SEC measurements and carbon balances in a Sturm test experiment. Based on these results degradation characteristics of potential oligomer intermediates resulting from a primary chain scission from copolyesters consisting of aromatic and aliphatic dicarbonic acids can be predicted depending on their composition. These results will have an evident influence on the evaluation of the biodegradability of commercially interesting copolyesters and lead to new ways of tailor-made designing of new biodegradable materials as well. 相似文献