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Gene therapy for spinal muscular atrophy:perspectives on the possibility of optimizing SMN1 delivery to correct all neurological and systemic perturbations

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Neural regeneration research 2025年第7期20卷 2011-2012页

作者： Sharon j.Brown rafael j.Yáñez-Muñoz Heidi r.Fuller School of Pharmacy and Bioengineering Keele UniversityKeeleUK Wolfson Center for Inherited Neuromuscular Disease TORCH BuildingRJAH Orthopaedic HospitalOswestryUK *** Center of Gene and Cell TherapyDepartment of Biological SciencesSchool of Life Sciences and the EnvironmentRoyal Holloway University of LondonEghamUK

Spinal muscular atrophy(SMA)is a genetic condition that results in selective lower motor neuron loss with concomitant muscle weakness and *** genetic cause of SMA was understood in 1995 when loss or impairment of the survival motor neuron 1(SMN1)gene was identified as the main contributing factor(Lefebvre et al.,1995).This,in combination with the discovery that humans have a“back-up”gene,SMN2,which can produce low levels(approximately 10%)of the full-length functional SMN protein,has led to the generation of SMA-specific gene *** was traditionally classified according to age of symptom onset and developmental milestones achieved,with life expectancy and severity varying between ***,SMN2 copy number is used as a proxy for the prediction of disease severity,with higher SMN2 copy number typically being associated with reduced severity of SMA,although this relationship is not absolute:some individuals with low SMN2 copy number have less severe SMA phenotypes and vice ***,the etiology of SMA is further complicated by other factors,such as non-typical nucleotide variants and SMN2-independent modifiers of disease severity.

关键词： atrophy traditionally absolute

德国GPS在线位移监控和预警系统工作原理与工程应用

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地质科技情报 2005年第B07期24卷 126-128页

作者：项伟 r.jger 中国地质大学工程学院武汉430074 德国卡尔斯鲁厄应用科技大学卡尔斯鲁厄76133

德国GPS在线位移监控和预警系统,除了可以进行GPS测量和数据处理外,还可给出观测点的实时三维位移数据、位移速度和位移加速度,绘出分析曲线,其划量精度达毫米级,并可根据预先设定的破坏概率自动报警。该系统可预测和预警自然灾害如滑... 详细信息

德国GPS在线位移监控和预警系统,除了可以进行GPS测量和数据处理外,还可给出观测点的实时三维位移数据、位移速度和位移加速度,绘出分析曲线,其划量精度达毫米级,并可根据预先设定的破坏概率自动报警。该系统可预测和预警自然灾害如滑坡、泥石流、地震等;在矿山开采作业中,可对矿坑、尾矿坝及采空区的地表塌陷进行监控;可用于岩土工程和土木工程建设中的高边坡、建筑物、基坑、挡土墙以及塔楼和桥梁高频率运动的监测;可广泛应用于水利工程建设,如大坝、大堤位移和沉陷的监控等。在道路建设中,可应用于隧道开挖、路堤沉陷和桥梁位移的观测。

关键词：德国GPS 在线监测位移预警系统大坝

Apples to oranges:environmentally derived,dynamic regulation of serotonin neuron subpopulations in adulthood?

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Neural regeneration research 2025年第9期20卷 2596-2597页

作者： Christopher.j.O’Connell Matthew j.robson Department of Genetics College of MedicineHarvard UniversityBostonMAUSA james L.Winkle College of Pharmacy Division of Pharmaceutical SciencesUniversity of CincinnatiCincinnatiOHUSA Neuroscience Graduate Program College of MedicineUniversity of CincinnatiCincinnatiOHUSA

Traumatic brain injury(TBI)is a public health problem with an undue economic burden that impacts nearly every age,ethnic,and gender group across the globe(Capizzi et al.,2020).TBIs are often sustained during a dynamic range of exposures to energetic environmental forces and as such outcomes are typically heterogeneous regarding severity and pathology(Capizzi et al.,2020).

关键词： sustained orange dynamic

Nuclear proceses in the continuum

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Nuclear proceses in the continuum

Temporal dynamics of neonatal hypoxic-ischemic encephalopathy injuries on magnetic resonance imaging

第十三届全国核结构研讨会暨第九次全国“核结构与量子力学”专题讨论会

作者： r.j.liotta KTH Alba Nova University CenterSE-10691 StockholmSweden

New experimental facilities make it possible to reach very unstable nuclei with properties which cannot be analysed without including the continuum part of the *** this talk a formalism suitable to treat such unstable... 详细信息

关键词： unstable nuclei continuum

来源： cnki会议评论

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Neural regeneration research 2025年第11期20卷 3144-3150页

作者： Holly Flyger Samantha j.Holdsworth Alistair.j.Gunn Laura Bennet Hamid Abbasi Mātai Medical research Institute Tairāwhiti GisborneNew Zealand Department of Anatomy&Medical Imaging Faculty of Medical and Health SciencesThe University of AucklandAucklandNew Zealand Center for Brain research The University of AucklandAucklandNew Zealand Auckland Bioengineering Institute The University of AucklandAucklandNew Zealand

Moderate to severe perinatal hypoxic-ischemic encephalopathy occurs in~1 to 3/1000 live births in high-income countries and is associated with a significant risk of death or neurodevelopmental *** assessment is important to help identify highrisk infants,to help families,and to support appropriate interventions.A wide range of monitoring tools is available to assess changes over time,including urine and blood biomarkers,neurological examination,and *** present,magnetic resonance imaging is unique as although it is expensive and not suited to monitoring the early evolution of hypoxic-ischemic encephalopathy by a week of life it can provide direct insight into the anatomical changes in the brain after hypoxic-ischemic encephalopathy and so offers strong prognostic information on the long-term outcome after hypoxic-ischemic *** review investigated the temporal dynamics of neonatal hypoxic-ischemic encephalopathy injuries,with a particular emphasis on exploring the correlation between the prognostic implications of magnetic resonance imaging scans in the first week of life and their relationship to long-term outcome prediction,particularly for infants treated with therapeutic hypothermia.A comprehensive literature search,from 2016 to 2024,identified 20 pertinent *** review highlights that while the optimal timing of magnetic resonance imaging scans is not clear,overall,it suggests that magnetic resonance imaging within the first week of life provides strong prognostic *** challenges limit the timing consistency,particularly the need for intensive care and clinical ***,although most reports examined the prognostic value of scans taken between 4 and 10 days after birth,there is evidence from small numbers of cases that,at times,brain injury may continue to evolve for weeks after *** suggests that in the future it will be important to explore a wider range of times after hypo

关键词： magnetic resonance imaging neonatal hypoxic-ischemic encephalopathy neurodevelopmental outcomes prognostic biomarkers in neuroimaging scan timing therapeutic hypothermia

Small scale biodiesel synthesis from waste frying oil and crude methanol in Morocco

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Chinese journal of Chemical Engineering 2016年第9期24卷 1178-1185页

作者： Fatiha Ouanji Marouane Nachid Mohamed Kacimi Leonarda F.liotta Fabtizio Puleo Mahfoud Ziyad Universite Mohammed V Faculte des SciencesDepartement de ChimieLaboratoire de Physico-chimie des Materiaux et Catalyse(URAC26)Avenue lbn BattoutaBP.1014RabatMorocco Hassan 11 Academy of Sdence and Technology RabatMorocco lstituto per lo Studio dei Materiali Nanostrutturati(ISMN)-CNr via Ugo La Malfa15390146 PalermoItaly

Biodiesel was produced at small scale by transesterification of used frying oil（UFO） recovered from Moroccan pastry shops and fish frying restaurants. Biodiesel was first synthesised at laboratory scale in order to optimize the transesterification parameters. The cost of the final product was also optimized using low-cost raw *** UFO and the produced biodiesel were characterized with several techniques including gas chromatography,1H NMr,13 C NMr, FTIr, and TGA–TDA techniques.1H NMr gas chromatographic analyses of the final product confirmed that the transesterification in the chosen experimental conditions was completed. These results were confirmed by TGA–TDA analysis used as new techniques to monitoring triglyceride conversion. The biodiesel did not contain any trace of glycerol, and it did meet the international standards. The transesterification at low cost in small scale conditions was performed at 60 ℃ using 1.2% of KOH and a methanol/oil molar ratio of 6：1. A yield of 80.8% was achieved. The properties of the produced biodiesel were found to be as good as those of biodiesels obeying to European standards. The biodiesel production was also performed at small-scale for individual utilisation. Thus, the product was tested in a kerosene stove for heating and non-modified commercial diesel engine producing electricity.

关键词： Biodiesel Transesterification Small-scale Thermogravimetry

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致病性奈瑟氏菌的动物模型

国外医学（微生物学分册） 1989年第6期12卷 263-265,266页

作者： Arko r.j 李自力

本文叙述了淋球菌和脑膜炎球菌的动物模型。淋球菌可用黑猩猩建立局限性尿道感染,非灵长类实验动物模型以近交系小鼠应用较广。豚鼠、家兔、大鼠及仓鼠均有一定的实用价值。脑膜炎球菌感染动物模型可使用猴、兔、豚鼠、小鼠及鸡胚,但以... 详细信息

本文叙述了淋球菌和脑膜炎球菌的动物模型。淋球菌可用黑猩猩建立局限性尿道感染,非灵长类实验动物模型以近交系小鼠应用较广。豚鼠、家兔、大鼠及仓鼠均有一定的实用价值。脑膜炎球菌感染动物模型可使用猴、兔、豚鼠、小鼠及鸡胚,但以各种近交系小鼠是为通用,小鼠腹腔内感染已用于检测脑膜炎球菌菌株变异和阐明代谢铁在脑膜炎感染中的作用,还能区别脑膜炎球菌不同毒力菌株。

关键词：奈瑟氏动物模型

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景观生态系统的基础研究

生态学进展 1989年第2期6卷 120-121,81页

作者： Boer,r.j 黄克新

水稻对氮素的吸收、分配及其在组织中的挥发损失

维普期刊数据库评论

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维普期刊数据库

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植物营养与肥料学报 2004年第6期10卷 579-583页

作者：黄见良邹应斌彭少兵 r.j Buresh 华中农业大学植物科技学院作物生理与栽培研究中心湖北武汉430070 湖南农业大学农学院湖南长沙410128 国际水稻研究所马尼拉

应用15N示踪技术研究了水稻不同生育期吸收的15N在各器官中的分配,以及后期植物组织中的挥发损失。结果发现,水稻在分蘖期吸收的氮量少于在幼穗分化期吸收的氮量;在分蘖期吸收的15N,标记结束时氮素主要分配于水稻的叶片中,至成熟期15N有... 详细信息

应用15N示踪技术研究了水稻不同生育期吸收的15N在各器官中的分配,以及后期植物组织中的挥发损失。结果发现,水稻在分蘖期吸收的氮量少于在幼穗分化期吸收的氮量;在分蘖期吸收的15N,标记结束时氮素主要分配于水稻的叶片中,至成熟期15N有39%转运至水稻子粒中;水稻在幼穗分化期吸收的15N,标记结束时氮素主要分配在水稻茎和叶鞘中,至成熟期15N有46%转运至水稻的子粒中;水稻在分蘖期和幼穗分化期吸收的氮素在后期可以通过植株组织挥发损失,至成熟期损失的比例分别达16 7%和13 4%。

关键词：水稻 ^15N示踪技术吸收分配氮素挥发损失