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1.
Summary Two female sibs are reported with a possibly new lethal malformation pattern, the major anomalies of which are: coarse face with small eyes and cloudy corneae, cleft soft palate, hypoplasia and absence of lobulation of both lungs, diaphragmatic defects, digitalisation of thumbs and distal limb deformities. 相似文献
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Cephas E. Small Sai‐Wing Tsang Song Chen Sujin Baek Chad M. Amb Jegadesan Subbiah John R. Reynolds Franky So 《Liver Transplantation》2013,3(7):909-916
Polymer bulk heterojunction solar cells based on low bandgap polymer:fullerene blends are promising for next generation low‐cost photovoltaics. While these solution‐processed solar cells are compatible with large‐scale roll‐to‐roll processing, active layers used for typical laboratory‐scale devices are too thin to ensure high manufacturing yields. Furthermore, due to the limited light absorption and optical interference within the thin active layer, the external quantum efficiencies (EQEs) of bulk heterojunction polymer solar cells are severely limited. In order to produce polymer solar cells with high yields, efficient solar cells with a thick active layer must be demonstrated. In this work, the performance of thick‐film solar cells employing the low‐bandgap polymer poly(dithienogermole‐thienopyrrolodione) (PDTG‐TPD) was demonstrated. Power conversion efficiencies over 8.0% were obtained for devices with an active layer thickness of 200 nm, illustrating the potential of this polymer for large‐scale manufacturing. Although an average EQE > 65% was obtained for devices with active layer thicknesses > 200 nm, the cell performance could not be maintained due to a reduction in fill factor. By comparing our results for PDTG‐TPD solar cells with similar P3HT‐based devices, we investigated the loss mechanisms associated with the limited device performance observed for thick‐film low‐bandgap polymer solar cells. 相似文献
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Song Q Pallikkuth S Bossuyt J Bers DM Robia SL 《The Journal of biological chemistry》2011,286(11):9120-9126
Na/K-ATPase (NKA) activity is dynamically regulated by an inhibitory interaction with a small transmembrane protein, phospholemman (PLM). Inhibition is relieved upon PLM phosphorylation. Phosphorylation may alter how PLM interacts with NKA and/or itself, but details of these interactions are unknown. To address this, we quantified FRET between PLM and its regulatory target NKA in live cells. Phosphorylation of PLM was mimicked by mutation S63E (PKC site), S68E (PKA/PKC site), or S63E/S68E. The dependence of FRET on protein expression in live cells yielded information about the structure and binding affinity of the PLM-NKA regulatory complex. PLM phosphomimetic mutations altered the quaternary structure of the regulatory complex and reduced the apparent affinity of the PLM-NKA interaction. The latter effect was likely due to increased oligomerization of PLM phosphomimetic mutants, as suggested by PLM-PLM FRET measurements. Distance constraints obtained by FRET suggest that phosphomimetic mutations slightly alter the oligomer quaternary conformation. Photon-counting histogram measurements revealed that the major PLM oligomeric species is a tetramer. We conclude that phosphorylation of PLM increases its oligomerization into tetramers, decreases its binding to NKA, and alters the structures of both the tetramer and NKA regulatory complex. 相似文献
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Zwinderman AH Glas AS Bossuyt PM Florie J Bipat S Stoker J 《Biostatistics (Oxford, England)》2008,9(3):513-522
We propose random-effects models to summarize and quantify the accuracy of the diagnosis of multiple lesions on a single image without assuming independence between lesions. The number of false-positive lesions was assumed to be distributed as a Poisson mixture, and the proportion of true-positive lesions was assumed to be distributed as a binomial mixture. We considered univariate and bivariate, both parametric and nonparametric mixture models. We applied our tools to simulated data and data of a study assessing diagnostic accuracy of virtual colonography with computed tomography in 200 patients suspected of having one or more polyps. 相似文献
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Aerial dispersal plasticity under different wind velocities in a salt marsh wolf spider 总被引:1,自引:0,他引:1
Dispersal can be regarded as a process operating both betweenand within patches of suitable habitat. For uncontrolled dispersalprocesses, the risk of crossing the borders of the habitat patchand arriving in the unsuitable landscape matrix will increasewith decreasing patch area, in particular when the distancebetween isolated habitat patches is larger than the species'average dispersal capacity. Ballooning dispersal in spiderscan be considered as a passive dispersal process, in which disperseddistances depend on the prevalent wind velocity. We executeda reaction norm analysis to analyze how dispersal propensityof the salt marsh wolf spider Pardosa purbeckensis dependedon population characteristics (patch size) and the environment(wind velocity). Dispersal propensity was affected by the interactionbetween wind velocity and maternal patch size. Ballooning propensitiesdecreased with decreasing salt marsh size. Interestingly, genotypesfrom large salt marshes show higher ballooning propensitiesunder higher wind velocities, whereas those from small habitatpatches show their highest dispersal propensity under low windvelocities. Crossing reaction norms and subsequently stronggenotype x environment interaction variation was observed inall populations but tended to be lower in genotypes from largesalt marshes. It is likely that this pattern results from differencesin wind velocityrelated costs of within-habitat dispersalin salt marshes of different sizes. 相似文献
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Inflow of seeds through the forest edge: evidence from seed bank and vegetation patterns 总被引:1,自引:0,他引:1
To determine the influence of the proximity of a forest edge on seed bank composition and diversity, we performed a seed bank sampling at ancient deciduous forests bordering intensive arable fields. Also vegetation patterns were taken into account. We hypothised that forest edges may facilitate the entrance of diaspores of invasive species into the forest and the successive incorporation of these species in the forest seed bank. We noticed a substantial influence of the proximity of an edge on seed bank composition at as well the forested side of the edge as the field side. The forest edge zone was limited to 3 m into the forest and the field edge zone extended 3m into the field. The seed bank samples of field and forest edge are characterised by a higher species diversity and seed density and a higher similarity between seed bank and vegetation, compared to field or forest samples. The forest edges contains fewer pioneer species in comparison with the forest interior and more competitive species and species of edges and clearings compared with field and forest samples. The seed longevity index increases towards the forest interior. We can conclude from our data that the forest and edge seed bank are composed by both seeds from recent dispersal processes and local seed set and by seeds originating from past vegetation on the site. Near the edge, actual seed input seems of primal importance. Further towards the forest interior seed input decreases and long-living seeds of past vegetation become more important. Ancient forest edges thus act as a barrier for seeds of species of the surrounding arable field. 相似文献
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Jeurissen A Bossuyt X 《Journal of immunology (Baltimore, Md. : 1950)》2004,172(5):2728; author reply 2728-2728; author reply 2729