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We describe twin girls with bilateral cerebrovascular disease. In one child, a diagnosis of moyamoya disease was made after presentation in infancy with an acute hemiparesis; her asymptomatic sibling was found to have significant bilateral cerebrovascular disease after neuropsychological evaluation and assessment with transcranial Doppler ultrasound. Both subjects showed a discrepancy between verbal and performance IQ and deficits on a test of frontal-lobe function suggesting that these domains should be targeted in cognitive assessment. Family members of subjects with moyamoya are at risk of cerebrovascular disease. Clinical symptoms do not reliably predict disease and those at risk should be offered screening with non-invasive vascular imaging.  相似文献   
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With the increasing use of electric motors in automobiles—in some cases as many as 100 electric motors per vehicle—alternatives to ferrite magnetic materials are under consideration. If more powerful magnetic materials could be implemented, size reductions in these motors could result in curb weight reductions that lead to improved fuel economy. NdFeB magnets are attractive as potential replacement materials, due to their higher flux density and thus smaller required size; however, high material costs have limited their implementation. To investigate the economic competitiveness of alternative magnetic materials, technical cost models (TCMs) were developed and used to examine the manufacturing economics related to the production of both NdFeB and ferrite magnets. Results from the TCMs are used to identify the cost drivers and opportunities for cost reductions. Subsequent investigations will include effects on vehicle fuel efficiency, and the environmental impact of magnet manufacture and disposal.  相似文献   
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The performance of step index up-tapers subjected to certain index perturbations in investigated by computer simulation. Concentric symmetric and asymmetric index profile perturbations as well as localized spherical, called regions bubbles, having a different index are studied. It is found that some low-valued perturbations of small scale size (less than a wavelength) do not alter the optical fiber performance. However, the amount of beam expansion in the up-taper may be ultimately limited by such perturbations. Excessive magnification in the presence of perturbations can cause a distortion of the quasi-Gaussian shape and symmetry of the pure step-index fundamental mode as well as increase the excess loss and degrade the lateral and axial displacement tolerance improvements of the up-tapers. For example, an index variation of Δn≈10-5 in conjunction with a perturbation scale size of 40 μm can be detrimental. Bubble-type perturbations could also cause similar problems. A single 12.8-μm-diameter bubble with a relative index of -0.02, embedded in a 128-μm-diameter step index core with relative index of 0.5%, produced a 0.3-dB excess loss  相似文献   
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In situ imaging techniques in conjunction with electrochemical measurements were used to study the behavior of steel surfaces in simple chloride solutions. The imaging method detected the presence of significantly thicker uniform layers than the original passive oxide. The layers were found to form in chloride after the steel was anodically polarized following reduction of the passive oxide film. The growth of the layer depended on the degree of prior passive oxide reduction. Formation of the layer was also sensitive to underlying structure and enabled rapid location of regions of higher corrosion rate across laser welds in steels.  相似文献   
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Anthocyanins in aged Cabernet Sauvignon red wines were studied by HPLC–MS. The major anthocyanin in 6, 7, and 8 year old red wine extracts was the dimer vitisin A produced by condensation of malvidin-3-O-glucoside mediated by pyruvic acid. In aged wine, the content of malvidin-3-O-glucoside decreased with age with a concomitant increase of vitisin A. The latter is accompanied by several condensation products with molecular weight up to 1500 m/z. Differential pulse voltammetry indicated that aged wines have lower antioxidant capacity than young wine (400 mV), but higher than malvidin-3-O-glucoside (483 mV). Tafel’s plots showed that the electrochemical process occurring in aged wines is different from young wines. Six, seven and eight year old wines show similar behaviour with plots of 234, 177 and 188 mV/dec, respectively. These values are higher than the expected 120 mV/dec corresponding to a first electronic transfer but smaller compared to the 523 mV/dec corresponding to young wine.  相似文献   
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Glyceraldehyde-3-phosphate dehydrogenase (GAPDH) expression is increased in Dunning R-3327 rat prostatic adenocarcinoma cell lines relative to normal rat ventral prostate tissue. GAPDH expression closely correlates with cell motility of Dunning prostate cancer cell lines and accurately distinguishes cell lines with high metastatic potential from those with low metastatic potential. Increased GAPDH expression in the cancer cell lines is not simply related to increased growth rate, since rapidly proliferating normal prostate tissue did not exhibit elevated GAPDH expression.  相似文献   
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We have analyzed the expression pattern of the Xenopus FGF-3 gene during early development and examined its biological activity in three different bioassays using Xenopus embryos. We show that from the early gastrula stage there is a domain of expression around the blastopore which becomes a posterior domain as the blastopore closes. An anterior ectodermal domain becomes detectable from mid-gastrula stages in the prospective hind-brain, and there are several later domains of expression: the midbrain-hindbrain junction, the otocyst, the pharyngeal pouches and the tailbud region. By using double whole-mount in situ hybridizations we show that the XFGF-3 expression in the brain is dynamically regulated both in time and space during development. The anterior domain of early neurula stage embryos corresponds to the prospective rhombomeres 3-5. By the time the neural tube is closed, XFGF-3 expression is restricted to r4 and later a new domain of expression is established at the midbrain/hindbrain junction. In addition, we show that, despite its difference in receptor specificity, XFGF-3 can induce the formation of mesoderm from animal caps similarly to other FGFs. It also displays a posteriorizing activity on whole embryos similar to other FGFs. Although the absence of maternal expression makes it unlikely that XFGF-3 is involved in mesoderm induction in vivo, its posterior domain of expression during gastrulation and its posteriorizing activity suggests that it participates in the maintenance of mesodermal gene expression and in the FGF mediated patterning of the anteroposterior axis during gastrulation.  相似文献   
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