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*** mediated PEX1 gene augmentation improves visual function in a mouse model for Zellweger spectrum disorder

Annals of Eye Science 2018年第1期 443-443页

作者： Catherine Argyriou Ji Yun Song Ania Polosa Bruno Cecyre jean-Francois Bouchard Pierre Lachapelle jean bennett Nancy Braverman Human Genetics McGill UniversityMontrealQCCanada Perelman School of Medicine University of PennsylvaniaPhiladelphiaPAUSA Ophthalomology McGill UniversityMontrealQCCanada School of Optometry University of MontrealMontrealQCCanada

Background:Zellweger spectrum disorder(ZSD)is an autosomal recessive disease caused by mutations in any one of 13 PEX genes whose protein products are required for peroxisome *** leading to blindness is one of the major handicaps faced by affected individuals,but treatment for this is supportive *** test whether we could improve visual function in ZSD,we performed a proof-of-concept trial for PEX1 gene augmentation therapy using the Pex1-G844D mouse model,which bears the equivalent to a common human *** model exhibits a gradual decline in scotopic ffERG response,an always residual photopic ffERG response,diminished visual acuity,and cone and bipolar cell ***:We administered subretinal injections of a PEX1-containing viral vector(***)to 2 mouse cohorts of 5 or 9 weeks of age.A GFP-containing vector was used as a control in the contralateral eye of each *** expression of the virus was confirmed by retinal histology/immunohistochemistry,and its ability to recover peroxisome import was confirmed in *** ffERG and optokinetic(OKN)analyses were performed on a subset of animals at 8,16,and 20 weeks after gene *** ffERG and OKN measures were performed when each cohort reached 32 weeks of age(23 or 27 weeks post injection).Results:Preliminary ffERG and OKN analyses at 8 weeks post injection showed mildly better retinal response and visual acuity,respectively,in the PEX1-injected eyes,as did ffERG analysis when each cohort reached 25 weeks of age(16 or 20 weeks after gene delivery).This effect was more pronounced in the cohort treated at 5 weeks of age,when ffERG response is highest in Pex1-G844D *** 32 weeks of age,the ffERG response in the PEX1-injected eyes was double that of GFP-injected eyes,on average,though there was no change in ***,in PEX1-injected eyes the photopic ffERG response improved over time,and the decline in scotopic b-wave amplitude was ameliorat

关键词： Retinal gene therapy peroxisome disorder Zellweger spectrum disorder(ZSD) PEX1

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Power Delivery Studies of Windbelt Generators with Different Architectures

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Journal of Energy and Power Engineering 2014年第11期8卷 1942-1951页

作者： David Eisen Kevin Zhang Linh Pham Fow-Sen Choa Nicole Armstrong Phillip Aye Ricardo Aye George bennett Robert Hawthorne Huguens jean Kimberly Brown Department of CSEE (Computer Science and Electrical Engineering) University of Maryland Baltimore County Baltimore MD21250 USA Long Reach High School Baltimore MD 21045 USA Global Outcomes Inc. Baltimore MD 21227 USA

Windbelt generators have been proposed as small, green power sources for battery charging applications. Some of the reported results lack detailed information about how key parameters influence the output power of the generator. In this work, we built prototypes with different architectures to study the voltage generation and power delivery as functions of belt tension, length, and electrical load at various wind speeds. We also studied the maximum power delivery conditions before the breakdown of the belt oscillation occurs. Our results are obtained from windbelt generators with two types of architectures： a conventional design with an adjustable belt that uses weight for tension control, and a revised design with a belt oscillation perpendicular to the coil axis. We have concluded that the breakdown of the belt oscillation at lower output resistances is a primary bottleneck that will limit windbelt systems to only very low power applications.

关键词： Windbelt generators aeroelastic flutter wind power green energy.

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Zn38Mg12Ca32Yb18大块金属玻璃的晶化动力学行为（英文）

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稀有金属材料与工程 2019年第5期48卷 1429-1434页

作者：乔吉超 Pelletier jean-Marc 姚尧葛渊西北工业大学陕西西安710072 Universite de Lyon MATEISUMR CNRS5510Bat.B.PascalINSA-LyonF-69621 Villeurbanne CedexFrance 西北有色金属研究院金属多孔材料国家重点实验室陕西西安710016

采用差示扫描量热分析仪,研究了Zn38Mg12Ca32Yb18大块金属玻璃的在等时和等温状态下的晶化动力学行为。在等时条件下,不同的理论模型来分析特征温度的名义激活能。结果表明,采用Kissinger模型,Flynn-Wall-Ozawa模型和Augis-bennett模型... 详细信息

采用差示扫描量热分析仪,研究了Zn38Mg12Ca32Yb18大块金属玻璃的在等时和等温状态下的晶化动力学行为。在等时条件下,不同的理论模型来分析特征温度的名义激活能。结果表明,采用Kissinger模型,Flynn-Wall-Ozawa模型和Augis-bennett模型计算的名义激活能相互吻合。再者,采用Johnson-Mehl-Avrami模型分析了等温过程中晶化转变动力学机。Avrami指数n的分布区间介于3.25到4.12。分析发现,在等温晶化过程中采用Arrhenius方程计算得到的激活能比在等时晶化过中采用Kissinger方程的激活能要大,这可能是由于等温退火过程中能垒比等时晶化过程中的要高所引起的。

关键词：金属玻璃差示扫描量热分析仪晶化动力学 Johnson-Mehl-Avrami模型 Avrami指数

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1D AND 2D CIRCUIT-CODE MODELING OF THE REFURBISHED Z ACCELERATOR

1D AND 2D CIRCUIT-CODE MODELING OF THE REFURBISHED Z ACCELER...

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The 17th International Conference on High-Power Particle Beams

作者： Kenneth W.Struve Lawrence F.bennett jean-Paul Davis Mark E.Savage David L.Smith William A.Stygar Timothy F.Chavez David D.Hinshelwood Tim C.Wagoner Sandia National Laboratories P.O.Box 5800AlbuquerqueNM 87185-1194USA EG&G 2420 Comanche Rd.NEAlbuquerqueNM87107 USA Naval Research Laboratory WashingtonDCUSA Ktech Corporation 10800 Gibson Blvd.SEAlbuquerqueNM87123USA

＜正＞Now that the refurbishment of the Z machine at Sandia is complete we are routinely operating with currents over 26 MA into various *** routine operation we are able to measure current and voltage throughout the machine and compare with circuit code predictions. These measurements have motivated improvements to the original model to include two-dimensional （2D） and three-dimensional （3D） *** this paper we describe a full-machine （36 module）,one-dimensional （1D） circuit model of Z using the Bertha code developed at the Naval Research *** also describe a model that uses 2D *** addition,we discuss plans for implementing a 3D circuit model to allow investigating azimuthal effects that can be important for tailoring the rise time,shape,and length of the Z output *** tailoring on Z is achieved by staggering the timing of the various pulsed-forming modules,and by operating individual modules in either long or short-pulse *** the individual lines combine at a large diameter and are separated azimuthally, transit-time effects can be important for determining transient field enhancements at the vacuum insulator and in the vacuum transmission lines,as well as pulse "shape. Finally,we compare the 1D and 2D models with data, and provide predictions for both short and long pulse modes for various load configurations.

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