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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

Typhoon parameter sensitivity of storm surge in the semi-enclosed Tokyo Bay

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Frontiers of Earth Science 2020年第3期14卷 553-567页

作者： Md.Rezuanul Islam Hiroshi Takagi school of environment and society Tokyo Institute of TechnologyTokyo152-8550Japan

In this study,a storm surge model of the semi-enclosed Tokyo Bay was constructed to investigate its hydrodynamic response to major typhoon parameters,such as the point of landfall,approach angle,forward speed,size,and *** typhoon simulation was validated for Typhoon Lan in 2017,and 31 hypothetical storm surge scenarios were generated to establish the sensitivity of peak surge height to the variation in typhoon *** maximum storm surge height in the upper bay adjacent to the Tokyo Metropolitan Area was found to be highly sensitive to the forward speed and size of the passing ***,the importance of these parameters in disaster risk reduction has been largely overlooked by researchers and disaster *** was also determined that of the many hypothetical typhoon tracks evaluated,the slow passage of a large and intense typhoon transiting parallel to the longitudinal axis of Tokyo Bay,making landfall 25 km southwest,is most likely to cause a hazardous storm surge scenario in the upper-bay *** results of this study are expected to be useful to disaster managers for advanced preparation against destructive storm surges.

关键词： storm surge risk semi-enclosed bay typhoon parameters parametric study Typhoon Lan

Short-fetch high waves during the passage of 2019 Typhoon Faxai over Tokyo Bay

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Frontiers of Earth Science 2022年第1期16卷 206-219页

作者： Hiroshi TAKAGI Atsuhei TAKAHASHI school of environment and society Tokyo Institute of TechnologyTokyo 152-8550Japan

The risk of wind waves in a bay is often overlooked,owing to the belief that peninsulas and islands will inhibit high ***,during the passage of a tropical cyclone,a semi-enclosed bay is exposed to twodirectional waves:one generated inside the bay and the other propagated from the outer *** Faxai in 2019 resulted in the worst coastal disaster in Tokyo Bay in the last few *** authors conducted a post-disaster survey immediately after this *** modeling was also performed to reveal the mechanisms of unusual high *** significant high-wave damage occurred on coasts facing the Pacific *** contrast,Fukuura-Yokohama,which faces Tokyo Bay,suffered overtopping waves that collapsed *** precisely reproduce multi-directional waves,the authors developed an extended parametric typhoon model,which was embedded in the JMA mesoscale meteorological model(JMA-MSM).The peak wave height was estimated to be 3.4 m off the coast of Fukuura,in which the contribution of the outer-sea waves was as low as 10%–20%.A fetchlimited wave developed over a short distance in the bay is considered the primary mechanism of the high *** maximum wave occurred on the left-hand side of the typhoon track in the bay,which appears to be contrary to the common understanding that it is safer within the semicircle of a storm than on the opposite *** Faxai was a small typhoon;however,if the radius was tripled,it is estimated that the wave height would exceed 3 m over the entire bay and surpass 4 m off the coasts of Yokohama and Chiba.

关键词： Typhoon Faxai Tokyo Bay high waves short fetch overtopping wave hindcasting parametric typhoon JMA-MSM storm surge

Combined reticular blind drainage and vertical hierarchical drainage system for landfills located in areas with high rainfall and high groundwater level

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Frontiers of environmental Science & Engineering 2016年第1期10卷 177-184页

作者： Wenjing LU Zhonge FU Yan ZHAO school of environment Tsinghua University Beijing 100084 China Loudi Civil and Architecture society Loudi 417000 China school of environment Beijing Normal University Beijing 100875 China

A novel water control technology that com- bines the features of a reticular blind drainage system and a vertical hierarchical drainage system is developed and applied in the Yanziyan Sanitary Landfill, which is located at an area （Loudi City, Hunan Province, China） with high rainfall and high groundwater level. The reticular blind drain system, which was installed on the bottom and side walls of the landfill site, can conveniently guide the flow of groundwater out of the site while preventing a disorga- nized flow of groundwater. The vertical hierarchical drainage system was installed to separate rainfall water and leachate in the landfill site, thus efficiently reducing the pressure of leachate treatment. The whole drainage system plays a key role in foundation stabilization by seepage control and separation and in the instant drainage of rainfall water. The leachate reduction efficiency of the drainage technology was calculated in terms of leachate production before （336519 m3） and after （29664 m3） technology application. Over 90% of leachate derived from rainfall water and groundwater inflow was avoided upon installation of the vertical hierarchical drainage and reticular blind drainage systems. The technology can thus be popularized and applied for water control in landfills located in areas with high rainfall and high groundwater level. The proposed technology can be used to alleviate the pressure of leachate treatment and to reduce the risk of instability.

关键词： landfill reticular blind drain vertical hier-archical drain guidance and drainage impermeable layer

Variable retention harvesting in Victoria’s Mountain Ash (Eucalyptus regnans) forests (southeastern Australia)

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Ecological Processes 2019年第1期8卷 12-20页

作者： David Lindenmayer David Blair Lachlan McBurney Fenner school of environment and society The Australian National UniversityCanberra ACT2601Australia

Variable retention harvesting is a silvicultural system that focuses on retaining key elements of stand structure at the time of logging and is increasingly being used *** describe the design and establishment of a variable retention harvesting experiment established in the Mountain Ash(Eucalyptus regnans)forests of the Central Highlands of Victoria,south-eastern *** experiment was instigated in 2003,and the work to date has shown that it has environmental benefits for certain groups of small mammals,birds,and vascular *** experiment has been integrated with an ongoing long-term monitoring program as well as other experiments such as those in post-fire salvage-logged ***,the results of various studies suggest that the potential value of variable retention harvesting extends beyond green-tree logging to post-fire salvage logging *** outline some of the challenges in,and new perspectives derived from,implementing and maintaining our *** included difficulties protecting islands from high-intensity post-harvest regeneration burns and threat of declining funding undermining ongoing project viability.A critically important perspective concerns the ecological and economic context in which variable retention harvesting is *** the particular case of Mountain Ash forests,assessments using formal IUCN criteria classify the ecosystem as being Critically Endangered under the Red Listed Ecosystem *** a result,Mountain Ash forests are at a high risk of ecosystem *** logging will increase that risk,making the basis for continued harvesting *** addition,economic analyses suggest that the value of natural assets,like water production,far outweigh the value of the wood products harvested from the Mountain Ash ecosystem,again leading to questions about the viability of ongoing *** therefore conclude that whilst variable retention harvesting has the potential to contrib

关键词： Alternative silvicultural systems Clear-cutting Biological legacies Forest biodiversity Forest economics,Retention islands

Prussian Blue Analogue‑Templated Nanocomposites for Alkali‑Ion Batteries:Progress and Perspective

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Nano-Micro Letters 2025年第1期17卷 216-261页

作者： Jian‑En Zhou Yilin Li Xiaoming Lin Jiaye Ye Key Laboratory of Theoretical Chemistry of environment Ministry of EducationSchool of ChemistrySouth China Normal UniversityGuangzhou 510006People’s Republic of China school of Chemistry and Physics Faculty of ScienceQueensland University of Technology2 George StreetBrisbaneQLD 4000Australia Centre for Materials Science Queensland University of Technology2 George StreetBrisbaneQLD 4000Australia

Lithium-ion batteries(LIBs)have dominated the portable electronic and electrochemical energy markets since their commercialisation,whose high cost and lithium scarcity have prompted the development of other alkali-ion batteries(AIBs)including sodium-ion batteries(SIBs)and potassium-ion batteries(PIBs).Owing to larger ion sizes of Na^(+)and K^(+)compared with Li^(+),nanocomposites with excellent crystallinity orientation and well-developed porosity show unprecedented potential for advanced lithium/sodium/potassium *** enticing open rigid framework structures,Prussian blue analogues(PBAs)remain promising self-sacrificial templates for the preparation of various nanocomposites,whose appeal originates from the well-retained porous structures and exceptional electrochemical activities after thermal *** review focuses on the recent progress of PBA-derived nanocomposites from their fabrication,lithium/sodium/potassium storage mechanism,and applications in AIBs(LIBs,SIBs,and PIBs).To distinguish various PBA derivatives,the working mechanism and applications of PBA-templated metal oxides,metal chalcogenides,metal phosphides,and other nanocomposites are systematically evaluated,facilitating the establishment of a structure–activity correlation for these *** on the fruitful achievements of PBA-derived nanocomposites,perspectives for their future development are envisioned,aiming to narrow down the gap between laboratory study and industrial reality.

关键词： Prussian blue analogues Self-sacrificial template Lithium-ion batteries Sodium-ion batteries Potassium-ion batteries

An ontology-based framework for extracting spatio-temporal influenza data using Twitter

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International Journal of Digital Earth 2019年第1期12卷 2-24页

作者： Udaya K.Jayawardhana Pece V.Gorsevski school of Earth Environment&SocietyBowling Green State UniversityBowling GreenOHUSA

Early detection of influenza outbreaks is one of the key priorities on a national level for preparedness and *** study presents the design and implementation of Fluwitter,which is a spatio-temporal webbased prototype framework for pseudo real-time detection of influenza outbreaks from ***,the framework integrates PostgreSQL database server with PostGIS spatial extension,Twitter streaming client,pre-processor,tagger and similarity calculator for semantic information extraction(IE).The IE of tagged terms is supported by Natural Language Processing(NLP)techniques,DBpediaSpotlight and WordNet Similarity for Java(WS4J),while data analytics,visualization,and mapping are supported by GeoServer and other GIS Free Open Source Software(FOSS).The prototype was calibrated to maximize detection of influenza using rules developed from ontology-based semantic similarity *** Twitter-generated influenza cases were validated by weekly hospitalization records issued by Ohio Department of Health(ODH).The optimized rule produced a final F-measure value of 0.72 and accuracy(ACC)value of 94.4%.The validation suggested the existence of moderate correlations for the beginning of the time period Southeast region(r=0.52),the Northwestern region(r=0.38),and the Central region(r=0.33)and weak correlations for the entire time *** potential strengths and benefits of the prototype are shown through spatio-temporal assessment and visualization of influenza potential in Ohio.

关键词： Tweet influenza social media geospatial analytics data mining web mapping

Radial variations in xylem sap flux in a temperate red pine plantation forest

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Ecological Processes 2021年第1期10卷 328-336页

作者： Alanna V.Bodo M.Altaf Arain school of Earth Environment and SocietyMcMaster UniversityHamiltonONCanada McMaster Centre for Climate Change HamiltonONCanada

Background:Scaling sap flux measurements to whole-tree water use or stand-level transpiration is often done using measurements conducted at a single point in the sapwood of the tree and has the potential to cause significant *** studies have shown that much of this uncertainty is related to(i)measurement of sapwood area and(ii)variations in sap flow at different depths within the tree ***:This study measured sap flux density at three depth intervals in the sapwood of 88-year-old red pine(Pinus resinosa)trees to more accurately estimate water-use at the tree-and stand-level in a plantation forest near Lake Erie in Southern Ontario,*** showed that most of the water transport(65%)occurred in the outermost sapwood,while only 26%and 9%of water was transported in the middle and innermost depths of sapwood,***:These results suggest that failing to consider radial variations in sap flux density within trees can lead to an overestimation of transpiration by as much as 81%,which may cause large uncertainties in water budgets at the ecosystem and catchment *** study will help to improve our understanding of water use dynamics and reduce uncertainties in sap flow measurements in the temperate pine forest ecosystems in the Great Lakes region and help in protecting these forests in the face of climate change.

关键词： Sap flow Transpiration Water use Red pine Temperate forest Great Lakes region

Hazards from lakes and reservoirs: new interpretation of the Vaiont disaster

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Journal of Mountain Science 2022年第6期19卷 1717-1737页

作者： Alan P.DYKES Edward N.BROMHEAD Centre for Engineering Environment and Society ResearchSchool of Engineering and the EnvironmentKingston UniversityPenrhyn RoadKingston upon ThamesKTi 2EEUK

Hazards in reservoirs and lakes arising from subaerial landslides causing impact waves(or ‘lake tsunamis’) are now well known, with several recent examples having been investigated in detail. The potential scale of such hazards was not widely known at the time of the Vaiont dam project in the 1950s and early 1960s, although a small wave triggered by a landslide at another new reservoir nearby in the Dolomites(northern Italy) drew the possible hazard to the attention of the Vaiont project’s managers. The Vaiont disaster in 1963 arose from a combination of disparate and seemingly unrelated factors and circumstances that led to an occurrence that could not have been imagined at that time. The ultimate cause was a very large landslide moving very rapidly into a reservoir and displacing the water. The resulting wave overtopped the dam to a height of around 175 m and around 2000 people were killed. This paper identifies and examines all of the issues surrounding the Vaiont dam and landslide in order to identify causal factors, contributory factors(including aggravating factors) and underlying factors. In doing so, it demonstrates that the disaster arose from the Vaiont dam project and cannot be attributed simply to the landslide. Underlying geological factors gave rise to the high speed of the landslide, which would have occurred anyway at some time. However, without the contributory factors that account for the presence of the reservoir, i.e. the choice of location for the project and management of the project with respect to a possible landslide hazard, there would have been no disaster. Indeed, the disaster could have been avoided if the reservoir could have been emptied pending further ground investigations. Understanding of this case provides many lessons for future dam projects in mountainous locations but also highlights an ongoing and perhaps under-appreciated risk from similar events involving other water bodies including geologically recent lakes formed behind n

关键词： Vaiont landslide Impulse wave Lake tsunami Dam project Causal factors for disaster