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351.
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Most spinal muscular atrophy (SMA) patients lack the survival motor neuron gene (SMN). However, the patients retain at least one copy of the cBCD541 gene (BCD), which is highly homologous with SMN. Here, we determined the SMN/BCD copy number ratios (the S/B ratios) of 12 parents of Japanese SMA patients with a homozygous SMN deletion, using competitive oligonucleotide priming polymerase chain reaction. We identified an S/B ratio of 2 in 25% of the parents examined, whereas less than 2% of parents of SMA patients in Western populations have an S/B ratio of 2. The high incidence of an S/B ratio of 2 in Japanese parents of SMA patients may reflect the characteristic genetic background of SMA in Japan.  相似文献   
353.
In this paper, we propose a MAP-Markov random field (MRF) based scheme for recovering the depth and the focused image of a scene from two defocused images. The space-variant blur parameter and the focused image of the scene are both modeled as MRFs and their MAP estimates are obtained using simulated annealing. The scheme is amenable to the incorporation of smoothness constraints on the spatial variations of the blur parameter as well as the scene intensity. It also allows for inclusion of line fields to preserve discontinuities. The performance of the proposed scheme is tested on synthetic as well as real data and the estimates of the depth are found to be better than that of the existing window-based depth from defocus technique. The quality of the space-variant restored image of the scene is quite good even under severe space-varying blurring conditions  相似文献   
354.
This paper presents a comprehensive state-of-the-art of the recent advances in simulation of power electronic converter systems. Knowing the importance of simulation, this paper reviews the various methods of modelling, circuit analysis approaches, numerical techniques etc. Several general purpose simulators and dedicated power electronic simulators have been discussed. A few demonstrative examples of simulation of power electronic converters by using different simulators are provided. Practical difficulties in simulation, challenges, new developments and scope for future work are also discussed.  相似文献   
355.
High-/spl kappa/ NMOSFET structures designed for enhancement mode operation have been fabricated with mobilities exceeding 6000 cm/sup 2//Vs. The NMOSFET structures which have been grown by molecular beam epitaxy on 3-in semi-insulating GaAs substrate comprise a 10 nm strained InGaAs channel layer and a high-/spl kappa/ dielectric layer (/spl kappa//spl cong/20). Electron mobilities of >6000 and 3822 cm/sup 2//Vs have been measured for sheet carrier concentrations n/sub s/ of 2-3/spl times/10/sup 12/ and /spl cong/5.85/spl times/10/sup 12/ cm/sup -2/, respectively. Sheet resistivities as low as 280 /spl Omega//sq. have been obtained.  相似文献   
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A study of forced convection film boiling on a horizontal isothermal plate is made using a treatment based on boundary layer theory. For the vapour, the velocity and temperature distributions are obtained from equations of motion and energy. For the liquid layer, the Howarth approximation for flow over a plate is used. Then, by employing interface matching conditions, the significance of the rhμ ratio between the vapour and the liquid on the variation of Nusselt number is examined.  相似文献   
360.
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