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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

Maximizing biomass utilization: An integrated strategy for coproducing multiple chemicals

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Journal of Energy Chemistry 2025年第1期100卷 180-191页

作者： Byeongchan Ahn Sua Jo Jonggeol Na J.Jay Liu Young-Ju Kim Wangyun Won department of Chemical and Biological Engineering Korea University department of Chemical Engineering (Integrated process) Kyung Hee University department of Chemical Engineering and Materials Science Ewha Womans University department of Chemical Engineering Pukyong National University Resources Engineering Plant Research department Korea Institute of Geoscience and Mineral Resources

Lignocellulosic biomass is one of the viable solutions to alleviate the global warming. However, the limited utilization of biomass majorly focused on cellulose and hemicellulose restricts the economic and environmental feasibilities. To cope with this issue, we proposed an integrated process of co-producing 1,6-hexanediol（1,6-HDO） with tetrahydrofuran and adipic acid from biomass, referred to as Strategy A. To compare the impacts of lignin upgrading and feedstock, Strategy B, which co-produces tetrahydrofuran alone, and Strategy C, which is the traditional route to produce 1,6-HDO from fossil fuels, were used. Heat networks are also designed to reduce operating costs and indirect carbon emissions due to energy consumption, saving 87% and 83% of the heat and cooling requirements, respectively, in Strategy A. The market competitiveness of Strategy A was evaluated by determining the minimum selling price through techno-economic analysis, and sustainability was thoroughly investigated by quantifying the environmental impacts through both midpoint and endpoint life-cycle assessments（LCAs）.Strategy A was found to be the most favorable both economically（USRDSCHARDOLLAR3,402/ton） and environmentally（-26.9 kg CO2eq.）. This indicates that lignin valorization is not only economically but also environmentally preferred. Finally, changes in economic and environmental feasibilities depending on economic, process, and environmental parameters were investigated using sensitivity and uncertainty analyses. The results of these analyses provide valuable insight into bio-based chemical production.

关键词： Biorefinery Carbon mitigation Climate change Global warming Lignin utilization Bioeconomy

Changes in Agricultural System as Farmers Adapt to Economic-Social and Climatic Changes in the Min Upriver Rural Areas in Western Sichuan,Southwestern China

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Journal of Mountain Science 2015年第3期12卷 747-758页

作者： ZHANG Long-jiang TANG Ya LIU Ben-hong department of environment College of Architecture and EnvironmentSichuan University

Rapid economic growth in China has brought about great economic-social changes in rural areas, having considerable impact on the society in economy and environment. With a per capita possession of about 0.08 ha of cropland, Chinese farmers in rural areas adopt various ways in response to these changes in a bit to maintain their livelihood, wherein the agricultural system is facing one more options possible. To understand how rural communities have used different mechanisms to adapt to the economic and natural changes, we joined a survey in dry valleys of the Min upriver area under Maoxian county of western Sichuan province, southwestern China and visited the local people. Changes in the main crop cultivation have shown up an important means to keep up their household income. Farm households start seeking economic growth through diversified cultivating of cereal and economic crops in five lines, namely cereal, apple monoculture, apple and vegetables, plum and vegetables, mixed fruits and vegetables. These new lines mirror farmers' flexibility to cope with today's economic-social and climatic changes. The farming operation has changed all the more from a subsistence on grain to special agricultural products. Economic reforms in the early 1980 s motivated theprogress first in conversion of production from grain to fruits, and the desire to increase family income turned out to be an impetus for the subsequent events. At present, more farmers moving out of the rural areas, uneasy availability of labor force, increased opportunity cost of labors and their wages, increased farm size, and the urgent demand for the agricultural labor force, all these combine into the trend of the agricultural system of China on facing further economic-social reforms and reconstruction of the countryside across China.

关键词： Agricultural system Climate change Farmers Economic impacts Social change MinRiver

Climate change and finger millet: Perception, trend and impact on yield in different ecological regions in Central Nepal

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Journal of Mountain Science 2019年第4期16卷 821-835页

作者： Dol Raj LUITEL Mohan SIWAKOTI Pramod Kumar JHA Central department of Botany Tribhuvan University department of environment Ministry of Forests and Environment

The views of local people on climate change along different ecological regions are relatively unexplored in Nepal. This study was conducted in 13 villages in central Nepal at different altitudes to document the views of small holder farmers and compare their perception with trends of climatic variables, finger millet yield, natural disasters, plant phenology(flowering and fruiting), status of forest and wild life, as well as the spread of diseases and pests. Analysis on the climatic data of stations for 36-41 years between 1975 and 2016 showed significant increases in the minimum temperature in lower tropical climatic region(<500 m), upper tropical to subtropical climatic region(500-2000 m) and temperate climatic region(2000-3000 m) by 0.01, 0.026 and 0.054℃/year, respectively, and an increase of maximum temperature by 0.008, 0.018, and 0.019℃/year, respectively. Rainfall showed a strongly significant decreasing trend in all elevation regions. This result matches with the views of respondents except 38% respondent from temperate climatic region. People from the temperate climatic region also mentioned that current onset of snowfall is delayed but amount of snowfall remained the same. From the documented records, except events of wild fire, frequency of natural disasters events have increased in the recent years, which was in harmony with the views of local people. Multi-linear regression analysis showed that contribution of climatic variables on finger millet yield in lower tropical climatic region and upper tropical to subtropical regions was 23% and 57.3%, respectively, which was supported by increasing trend on average growing degree day(GDD) temperature at the rate of 0.01℃ in upper tropical to subtropical region and 0.007℃ in lower tropical climatic region yearly. Finger millet yield has been increasing at the rate of 7.39 and 36.9 kg/ha yearly in lower tropical climatic region and upper tropical to subtropical climatic region, respectively. This result provide

关键词： Climate change Growing degree days (GDD) Natural disaster Phenology Precipitation Small holder farmers

Using fluorescence surrogates to track algogenic dissolved organic matter(AOM) during growth and coagulation/flocculation processes of green algae

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Journal of environmental Sciences 2019年第5期31卷 311-320页

作者： Quang Viet Ly Mi-Hee Lee Jin Hur department of environment & Energy Sejong University

Tracking the variation of the algogenic organic matter(AOM) released during the proliferation of green algae and subsequent treatment processes is crucial for constructing and optimizing control strategies. In this study, the potential of the spectroscopic tool was fully explored as a surrogate of AOM upon the cultivation of green algae and subsequent coagulation/flocculation(C/F) treatment processes using ZrCl_4 and Al_2(SO_4)_3. Fluorescence excitation emission matrix coupled with parallel factor analysis(EEM-PARAFAC) identified the presence of three independent fluorescent components in AOM, including protein-like(C1), fulvic-like(C2) and humic-like components(C3). Size exclusion chromatography(SEC) revealed that C1 in AOM was composed of large-sized proteins and aromatic amino acids. The individual components exhibited their unique characteristics with respect to the dynamic changes. C1 showed the highest correlation with AOM concentrations(R^2= 0.843) upon the C/F processes. C1 could also be suggested as an optical predictor for the formation of trihalomethanes upon the C/F processes. This study sheds a light for the potential application of the protein-like component(C1) as a practical surrogate to track the evolution of AOM in water treatment or wastewater reclamation systems involving Chlorella vulgaris green algae.

关键词： EEM-PARAFAC Enhanced coagulation Size exclusion chromatography Algal dissolved organic matter Trihalomethanes

Importance of nutrient availability for soluble microbial products formation during a famine period of activated sludge:Evidence from multiple analyses

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Journal of environmental Sciences 2019年第10期31卷 112-121页

作者： Tahir Maqbool Jinwoo Cho Jin Hur department of environment and Energy Sejong University

Much remains unknown about compositional variations in soluble microbial products(SMP)with the shift of the substrate condition from a feast to a famine phase in biological treatment *** study demonstrated that the formation of SMP could be suppressed by up to 75%during the famine phase with the addition of essential *** contrast,presence of electron acceptor did not play any significant role during the stress condition,showing the similar amounts of SMP(r=0.98,p0.95).The fluorescence excitation-emission matrix coupled with parallel factor analysis(EEM-PARAFAC)revealed the presence of four different fluorescent components,including two protein-like(C1 and C4),fulvic-like(C2),and humic-like(C3)components,in the SMP and bEPS formed at different *** C1 and C4 showed increasing trends(R^2>0.95)with the length of starvation in the bioreactors without essential *** availability was found to be a key factor to quench the production of large-sized *** study provides a wealth of information on operation conditions of activated sludge treatment systems to minimize large sized SMP molecules(particularly proteins),which typically exert many environmental concerns to effluent organic matter quality.

关键词： Soluble microbial products Nutrient availability EEM-PARAFAC SEC-OCD-OND Famine phase

Status and prospects for symmetric organic redox flow batteries

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Journal of Energy Chemistry 2025年第1期100卷 125-143页

作者： Md Al Raihan C.Adam Dyker department of Chemistry University of New Brunswick

As environmental concerns from fossil fuel consumption intensify, large-scale energy storage becomes imperative for the integration of renewable sources like wind, hydro, and solar with the electrical *** flow batteries, particularly those employing organic molecules, are positioned as a key technology for this purpose. This review explores the growing field of symmetric organic redox flow batteries(ORFBs) within this context. Unlike traditional asymmetric designs based on unique active materials for each electrode, symmetric ORFBs involve a single bipolar species for both electrodes. This review highlights the benefits of a symmetric design, and categorizes five distinct classes of organic bipolar molecules used in both aqueous and non-aqueous solvents. By providing a comprehensive overview of their cell cycling and performance characteristics, the strengths and weaknesses of the diverse categories of bipolar molecules are highlighted for both solvent systems, as are opportunities for future development. This should guide new research directions and advance the development of practical symmetric ORFBs.

关键词： Redox flow battery Symmetric battery Organic battery Aqueous electrolyte Non-aqueous electrolyte

Baptism by fire: the pivotal role of ancient conflagrations in evolution of the Earth's flora

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National Science Review 2018年第2期5卷 237-254页

作者： Tianhua He Byron B.Lamont department of environment and Agriculture Curtin University

Fire became a defining feature of the Earth's processes as soon as land plants evolved 420 million years ago and has played a major role in shaping the composition and physiognomy of many ecosystems ever ***, there remains a general lack of appreciation of the place of fire in the origin, evolution, ecology and conservation of the Earth's biodiversity. We review the literature on the presence of fire throughout the Earth's history following the evolution of land plants and examine the evidence for the origin and evolution of adaptive functional traits, biomes and major plant groups in relation to fire. We show that:(1) fire activities have fluctuated throughout geological time due to variations in climate, and more importantly in atmospheric oxygen, as these affected fuel levels and flammability;(2) fire promoted the early evolution and spread of major terrestrial plant groups;(3) fire has shaped the floristics, structure and function of major global biomes; and(4) fire has initiated and maintained the evolution of a wide array of fire-adapted functional traits since the evolution of land plants. We conclude that fire has been a fundamental agent of natural selection on terrestrial plants throughout the history of life on the Earth's land surface. We suggest that a paradigm shift is required to reassess ecological and evolutionary theories that exclude a role for fire,and also there is a need to review fire-suppression policies on ecosystem management and biodiversity conservation in global fire-prone regions.

关键词： angiosperms ecosystems fossil charcoal palaeo-atmospheric oxygen terrestrial floras wildfire

Impacts of reforestation on woody species composition,species diversity and community structure in dry-hot valley of the Jinsha River,southwestern China

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Journal of Mountain Science 2016年第12期13卷 2182-2191页

作者： GONG Zhi-lian TANG Ya department of environment College of Architecture and Environment Siehuan University Chengdu 510065 China department of environment Engineering College of Energy and Environment Xihua University Chengdu 610039 China

To understand the impacts of reforestation on woody species composition,species diversity and community structure,seven plantation forests in dryhot valley of the Jinsha River in Southwest China were investigated,with adjacent wastelands,natural shrub grassland and a natural forest as *** importance value,species richness,species heterogeneity and Sorenson similarity index between plantations and the natural forest were *** indicated that compared to wastelands and natural shrub grassland,reforestation improved species diversity and community structure,and more forest woody species found suitable habitats in *** diversity in understory of plantations and Sorenson similarity index were significantly negatively correlated with stem density in mature plantations(26-31 years old).Higher species diversity and Sorenson similarity index existed in mature sparse plantations due to lower stem density and more tree species planted *** contrast,reference natural forest,with species heterogeneity of 2.28 for shrub layer,showed the highest species *** would take a long time for species composition and diversity to recover through reforestation in a dry-hot ***,it was essential to protect remnant natural forests strictly and reforest with suitable management such as lower stem density and increasing genetic diversity of trees planted.

关键词： Biodiversity Plantations Dry hot valley Genetic resources Natural forest Reforestation