检索结果-南通市图书馆

Heterozygous carriers of galactocerebrosidase mutations that cause Krabbe disease have impaired microglial function and defective repair of myelin damage

引用

Neural Regeneration Research 2018年第3期13卷 393-401页

作者： nicole j.scott-hewitt Christopher j.Folts Mark D.Noble Department of Biomedical Genetics University of Rochester School of Medicine and Dentistry

This review addresses two puzzling findings related to mutations in galactocerebrosidase （GALC） that cause Krabbe disease （KD）, a severe lysosomal storage disorder characterized by extensive myelin damage in children with mutations in both GALC alleles. First, heterozygous carriers of KD-causing mutations, which include the biological parents of children with KD, exhibit increased risk for developing other diseases. Second, variants in the GALC locus increase the risk of developing multiple sclerosis （MS）, another disease characterized by extensive myelin damage. What explains these correlations？ In studies on cuprizone-induced myelin damage in heterozygous （GALC＋/–） mice carrying one copy of a mutation that causes KD-like disease, the extent of damage was similar in GALC＋/– and wild-type （WT） mice. In contrast, GALC＋/- mice had striking defects in repair of cuprizone-induced damage. We further found unexpected microglial defects in myelin debris clearance and in the ability to up-regulate the Trem2 microglial protein critical for debris uptake. These defects were rescued by exposure to a lysosomal re-acidifying drug discovered in our studies on KD, and which provides multiple clinically relevant benefits in the twitcher （GALC＋/–） mouse model of KD. Thus, heterozygous GALC mutations cause effects on biological function that may help to understand the increased disease risk in heterozygous carriers of such mutations and to understand why GALC variations increase the risk of MS. Our findings indicate that while some genetic risk factors may contribute to complex diseases by increasing the risk of tissue damage, others may do so by compromising tissue repair.

关键词： vulnerability locus multiple sclerosis Krabbe disease galactocerebrosidase microglia myelin repair lysosomal storage disorders heterozygous carriers Trem2 demyelination

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Mitochondrial therapeutics and mitochondrial transfer for neurodegenerative diseases and aging

引用

Neural Regeneration Research 2025年第3期20卷 794-796页

作者： Neville Ng Michelle Newbery nicole Miles Lezanne Ooi Molecular Horizons and School of Chemistry and Molecular Bioscience University of WollongongWollongongNSWAustralia

Mitochondrial dysfunction and neurodegeneration:Progressive neurodegenerative diseases affect a significant proportion of the population;in a single year,there are as many as 276 million disabilities and 9 million dea... 详细信息

关键词： degenerative diseases Mitochondrial

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

SEISMIC RANDOM VIBRATION ANALYSIS OF STOCHASTIC STRUCTURES USING RANDOM FACTOR METHOD

引用

Chinese journal of Mechanical Engineering 2006年第1期19卷 1-8页

作者： KESSISSOGLOU nicole j School of Mechanical and Manufacturing Engineering

Seismic random vibration analysis of stochastic truss structures is presented. A new method called random factor method is used for dynamic analysis of structures with uncertain parameters, due to variability in their material properties and geometry. Using the random factor method, the natural frequencies and modeshapes of a stochastic structure can be respectively described by the product of two parts, corresponding to the random factors of the structural parameters with uncertainty, and deterministic values of the natural frequencies and modeshapes obtained by conventional finite element analysis. The stochastic truss structure is subjected to stationary or non-stationary random earthquake excitation. Computational expressions for the mean and standard deviation of the mean square displacement and mean square stress are developed by means of the random variable＇s functional moment method and the algebra synthesis method. An antenna and a truss bridge are used as practical engineering examples to illustrate the application of the random factor method in the seismic response analysis of random structures under stationary or non-stationary random earthquake excitation.

关键词： Seismic random vibration Uncertainty structures Random factor methodNon-stationary ground motion

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Silk-based nerve guidance conduits with macroscopic holes modulate the vascularization of regenerating rat sciatic nerve

引用

Neural Regeneration Research 2025年第6期20卷 1789-1800页

作者： Carina Hromada Patrick Heimel Markus Kerbl LászlóGál Sylvia Nürnberger Barbara Schaedl james Ferguson nicole Swiadek Xavier Monforte johannes C.Heinzel Antal Nógrádi Andreas H.Teuschl-Woller David Hercher Department Life Science Engineering University of Applied Sciences Technikum WienViennaAustria Austrian Cluster for Tissue Regeneration ViennaAustria Ludwig Boltzmann Institute for Traumatology The Research Center in Cooperation with AUVAViennaAustria University Clinic of Dentistry Medical University of ViennaViennaAustria Department of Plastic Reconstructive and Aesthetic SurgeryLandesklinikum Wiener Neustadt2700 Wiener NeustadtAustria Department of Anatomy Histology and EmbryologyAlbert Szent-Györgyi Medical SchoolUniversity of SzegedSzegedHungary Medical University of Vienna Department of Orthopedics and Trauma SurgeryDevision of Trauma SurgeryViennaAustria Department of Hand- PlasticReconstructive and Burn SurgeryBG Unfallklinik TuebingenUniversity of TuebingenTuebingenGermany

Peripheral nerve injuries induce a severe motor and sensory deficit. Since the availability of autologous nerve transplants for nerve repair is very limited, alternative treatment strategies are sought, including the use of tubular nerve guidance conduits(tNGCs). However, the use of tNGCs results in poor functional recovery and central necrosis of the regenerating tissue, which limits their application to short nerve lesion defects(typically shorter than 3 cm). Given the importance of vascularization in nerve regeneration, we hypothesized that enabling the growth of blood vessels from the surrounding tissue into the regenerating nerve within the tNGC would help eliminate necrotic processes and lead to improved regeneration. In this study, we reported the application of macroscopic holes into the tubular walls of silk-based tNGCs and compared the various features of these improved silk^(+) tNGCs with the tubes without holes(silk^(–) tNGCs) and autologous nerve transplants in an 8-mm sciatic nerve defect in rats. Using a combination of micro-computed tomography and histological analyses, we were able to prove that the use of silk^(+) tNGCs induced the growth of blood vessels from the adjacent tissue to the intraluminal neovascular formation. A significantly higher number of blood vessels in the silk^(+) group was found compared with autologous nerve transplants and silk^(–), accompanied by improved axon regeneration at the distal coaptation point compared with the silk^(–) tNGCs at 7 weeks postoperatively. In the 15-mm(critical size) sciatic nerve defect model, we again observed a distinct ingrowth of blood vessels through the tubular walls of silk^(+) tNGCs, but without improved functional recovery at 12 weeks postoperatively. Our data proves that macroporous tNGCs increase the vascular supply of regenerating nerves and facilitate improved axonal regeneration in a short-defect model but not in a critical-size defect model. This study suggests that further optimizatio

关键词： axon regeneration blood vessel functional recovery macroporous nerve lesion peripheral nerve repair sciatic nerve silk-based nerve guidance conduit vascularization

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Forecasting the development of boreal paludified forests in response to climate change: a case study using Ontario ecosite classification

引用

Forest Ecosystems 2015年第1期2卷 45-55页

作者： Benoit Lafleur nicole j Fenton Yves Bergeron Institut de recherche sur les forêts Université du Québec en Abitibi-Témiscamingue 445 boul. de l’Université Rouyn-Noranda QC J9X 5E4 Canada Centre d’étude de la forêt Université du Québec à Montréal 141 Avenue du Président-Kennedy Montréal QC H2X 1Y4 Canada

Background：Successional paludification,a dynamic process that leads to the formation of peatlands,is influenced by climatic factors and site features such as surficial deposits and soil *** boreal regions,projected climate change and corresponding modifications in natural fire regimes are expected to influence the paludification process and forest *** objective of this study was to forecast the development of boreal paludified forests in northeastern North America in relation to climate change and modifications in the natural fire regime for the period 2011–***：A paludification index was built using static（*** deposits and soil texture）and dynamic（*** regime and soil organic layer thickness）stand scale factors available from forest *** index considered the effects of three temperature increase scenarios（i.e.＋1°C,＋3°C and＋6°C）and progressively decreasing fire cycle（from 300 years for 2011–2041,to 200 years for 2071–2100）on peat accumulation rate and soil organic layer（SOL）thickness at the stand level,and paludification at the landscape ***：Our index show that in the context where in the absence of fire the landscape continues to paludify,the negative effect of climate change on peat accumulation resulted in little modification to SOL thickness at the stand level,and no change in the paludification level of the study area between 2011 and ***,including decreasing fire cycle to the index resulted in declines in paludified ***,the index predicts a slight to moderate decrease in the area covered by paludified forests in 2100,with slower rates of ***：Slower paludification rates imply greater forest productivity and a greater potential for forest harvest,but also a gradual loss of open paludified stands,which could impact the carbon balance in paludified ***,as the thick Sphagnum layer typical of paludified forests may protect soil organic layer from dr

关键词： Boreal forest Canada Carbon sequestration Paludification Peat forests Forest harvest

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Micro-arcsecond Celestial Reference Frames:definition and realization——Impact of the recent IAU Resolutions

引用

Research in Astronomy and Astrophysics 2012年第8期12卷 1162-1184页

作者： nicole Capitaine SYRTE Observatoire de ParisCNRSUPMC61 avenue de l’Observatoire75014ParisFrance

The adoption of the International Celestial Reference System （ICRS）, based on Very Long Baseline Interferometry （VLBI） observations of extragalactic radiosources by the International Astronomical Union （IAU） since 1998 january 1, opened a new era for astronomy. The ICRS and the corresponding frame, the International Celestial Reference Frame （ICRF）, replaced the Fundamental Catalog （FK5） based on positions and proper motions of bright stars, with the Hipparcos cat- alog being adopted as the primary realization of the ICRS in optical wavelengths. According to its definition, the ICRS is such that the barycentric directions of distant extragalactic objects show no global rotation with respect to these objects; this pro- vides a quasi-inertial reference for measuring the positions and angular motions of the celestial objects. Other resolutions on reference systems were passed by the IAU in 2000 and 2006 and endorsed by the International Union of Geodesy and Geophysics （IUGG） in 2003 and 2007, respectively. These especially concern the definition and re- alization of the astronomical reference systems in the framework of general relativity and transformations between them. First, the IAU 2000 resolutions refined the con- cepts and definition of the astronomical reference systems and parameters for Earth＇s rotation, and adopted the IAU 2000 precession-nutation. Then, the IAU 2006 resolutions adopted a new precession model that is consistent with dynamical theories; they also addressed definition, terminology or orientation issues relative to reference systems and time scales that needed to be specified after the adoption of the IAU 2000 resolutions. An additional IUGG 2007 resolution defined the International Terrestrial Reference System （ITRS） so that it strictly complies with the IAU recommendations. Finally, the IAU 2009 resolutions adopted a new system of astronomical constants and an improved realization of the ICRF. These fundamental changes have led to significant

关键词： astrometry and celestial mechanics: astrometry -- reference systems --Earth -- techniques: interferometric

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Winter is coming:care of the febrile children in the time of COVID-19

引用

World journal of Pediatrics 2021年第1期17卷 6-7页

作者： nicole Gerber jonathan S.Farkas Adam j.Ratner Weill Cornell Medical Center New York Presbyterian525 East 68th StreetM-131New YorkNY 10021USA Department of Emergency Medicine Division of Pediatric Emergency Medicine525 East 68th StreetM130New YorkNY 10021USA New York University Langone Health/Bellevue Hospital Center 462 First AveNew YorkNY 10016USA

In the pediatric emergency department(PED),winter seems to arrive earlier each *** the broken bones and lac-erations of the summer,the winter season brings fevers and respiratory *** is our bread and butter-it is the most common reason for PED visits[1].Fever phobia is ingrained in our society[2].In the pre-vaccine era,a febrile child was cause for concern;those who were ill-appearing had high rates of bacterial meningitis,and even well-appear-ing children could not be rapidly *** bac-teremia was a common condition in well-appearing young febrile children and frequently required laboratory evalua-tion and treatment with empiric antibiotics[3].

关键词： febrile winter children

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Male obesity and subfertility, is it really about increased adiposity？

引用

Asian journal of Andrology 2015年第3期17卷 450-458页

作者： nicole O McPherson Discipline of Obstetrics and Gynaecology School of Paediatrics and Reproductive Health The Robinson Institute The University of Adelaide South Australia 5005 Australia Freemasons Foundation Center for Mens Health The University of Adelaide South Australia 5005 Monash IVF Group Melbourne Victoria 3168 Australia

The prevalence of overweight and obesity in reproductive-aged men is increasing worldwide, with 〉70% of men 〉18 years classified as overweight or obese in some western nations. Male obesity is associated with male subfertility, impairing sex hormones, reducing sperm counts, increasing oxidative sperm DNA damage and changing the epigenetic status of sperm. These changes to sperm function as a result of obesity, are further associated with impaired embryo development, reduced live birth rates and increased miscarriage rates in humans. Animal models have suggested that these adverse reproductive effects can be transmitted to the offspring; suggesting that men＇s health at conception may affect the health of their children, in addition to higher adiposity, male obesity is associated with comorbidities, including metabolic syndrome, hypercholesterolemia, hyperleptinemia and a pro-inflammatory state, all which have independently been linked with male subfertility. Taken together, these findings suggest that the effects of male obesity on fertility are likely multifactorial, with associated comorbidities also influencing sperm, pregnancy and subsequent child health.

关键词： embryo fertility infertility inflammation metabolic syndrome sperm

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Anticipated Inversion and Visibility Conditions over Glacier Bay with a Changing Climate

引用

journal of Environmental Protection 2015年第5期6卷 515-537页

作者： nicole Molders scott Gende Department of Atmospheric Sciences Geophysical Institute and College of Natural Science and MathematicsUniversity of Alaska FairbanksFairbanksUSA National Park Service Glacier Bay Field StationJuneauUSA

A RCP4.5 simulation from the Community Earth System Model was downscaled by the Weather Research and Forecasting Model, inline coupled with chemistry, to examine how climate change may affect inversions and visibility in Glacier Bay in the presence of cruise-ship visitations. Mean downscaled climate conditions for the tourist seasons for 2006-2012 were compared with downscaled conditions for 2026-2032 with identical cruise-ship entries and operating conditions thereby isolating pollutant retention and visibility differences caused by atmospheric climate change. Notable changes in future temperature, humidity, precipitation, and wind-speed occurred for large areas of Southeast Alaska and the Gulf of Alaska, although the anticipated differences were less pronounced in Glacier Bay due to the presence of the large glaciers and ice fields. While increased sensible heat and water vapor in the atmospheric boundary layer contributed to on average 4.5 h reduced inversion duration in Glacier Bay, the on average 0.23 m·s-1 reduced wind speeds increased inversion frequency by 4% on average. The future on average wetter conditions and altered precipitation patterns in Glacier Bay affected the removal of gases and particulate matter emitted by cruise ships locally or advected from areas outside the park. Season-spatial averaged visibility in Glacier Bay remained the same. However, visibility was degraded in the future scenario later in the season and slightly improved during spring. The warmer conditions contributed to decreased visibility indirectly by tieing up less NO2 in PAN and increasing biogenic NOx emissions. The wetter conditions contributed to reduced visibility in the last third of the tourist season.

关键词： Glacier Bay National Park Inversions Visibility CESM WRF/Chem Downscaling

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Impacts of Cruise-Ship Entry Quotas on Visibility and Air Quality in Glacier Bay

引用

journal of Environmental Protection 2015年第11期6卷 1236-1256页

作者： nicole Molders scott Gende Department of Atmospheric Sciences Geophysical Institute and College of Natural Science and MathematicsUniversity of Alaska FairbanksFairbanksAKUSA Glacier Bay Field Station National Park ServiceJuneauAKUSA

Managers at Glacier Bay National Park must annually determine the allowable number of cruise-ship entries into the park. This decision considers how differences in visitor volume may affect park resources. This study quantified the impacts to air quality and visibility under different ship quotas using simulations with the Weather Research and Forecasting model inline coupled with chemistry. Results of the simulation assuming two entries per day for May 15 to September 15, 2008 (QTA;248 ship entries representing a 35% increase) were compared to those of the 2008 cruise-ship activity (REF;184) during that timeframe. A simulation without anthropogenic emissions (CLN) served to assess the overall impacts of cruise-ship emissions on visibility and air quality in Glacier Bay. Compared to REF, the increased entry quotas shifted chemical regimes and aerosol composition, depending upon thermodynamical conditions, and ambient concentrations. On days with notable regime shifts, sulfur-dioxide concentrations deceased while ammonium-sulfate aerosol concentrations increased. The increased quotas also altered the fine-to-coarse aerosol ratios in both directions despite constant ratio of fine-to-coarse aerosol emissions. In Glacier Bay, the days with worst visibility coincided with high relative humidity, although this relationship varied by scenario. On the 20% worst days, mean visibility was slightly better in CLN (mean haze index over Glacier Bay waters = 2.9 dv) than in REF ( = 3.1 dv). While increased emissions in QTA reduced mean visibility by 0.1 dv, the 10th, 50th and 90th percentile of haze indices remained identical to those in REF. Best (worst) visibility occurred on the same days in REF and QTA due to emission impacts, but on different days than in CLN because relative humidity solely governed visibility in CLN. While calm wind played no role for visibility in CLN, wind speed gained similar importance for visibility as relative humidity in REF and QTA. Overall, increas

关键词： Glacier Bay National Park Visibility Air Quality WRF/Chem Cruise-Ship Emission Impacts in Pristine Areas

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

欢迎您,

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案：

请选择收藏分类：

通借通还

欢迎您,

建议与咨询 留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案： 新增检索档案 确定 取消

请选择收藏分类： 新增自定义分类 确定 取消

通借通还

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

请选择保存的检索档案：

请选择收藏分类：