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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

Heterotrophic ammonium removal characteristics of an aerobic heterotrophic nitrifying-denitrifying bacterium, Providencia rettgeri YL

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journal of environmental Sciences 2009年第10期21卷 1336-1341页

作者： taylor Shauna M School of environmental Science and engineering Shanghai Jiao Tong University

Bacterium Providencia rettgeri YL was found to exhibit an unusual ability to heterotrophically nitrify and aerobically denitrify various concentrations of ammonium （NH4^＋-N）. In order to further understand its removal ability, several experiments were conducted to identify the growth and ammonium removal response at different carbon to nitrogen （C/N） mass ratios, shaking speeds, temperatures, ammonium concentrations and to qualitatively verify the production of nitrogen gas using gas chromatography techniques. Results showed that under optimum conditions （C/N 10, 30℃, 120 r/min）, YL can significantly remove low and high concentrations of ammonium within 12 to 48 h of growth, respectively. The nitrification products hydroxylamine （NHzOH）, nitrite （NO2^-） and nitrate （NO3^-） as well as the denitrification product, nitrogen gas （N2）, were detected under completely aerobic conditions.

关键词： Providencia rettgeri YL heterotrophic ammonium removal aerobic denitrification

Post Locoregional Therapy Treatment Imaging in Hepatocellular Carcinoma Patients:A Literature-based Review

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journal of Clinical and Translational Hepatology 2018年第2期6卷 189-197页

作者： Shamar Young Andrew j.taylor Tina Sanghvi Department of Radiology University of MinnesotaMinneapolisMNUSA

Imaging plays a crucial role in the diagnosis of hepatocellular carcinoma(HCC)as well as in determining treatment efficacy,or complications,following *** other cancers,HCC is most commonly treated by locoregional therapies(LRTs)such as thermal ablation,transarterial chemoembolization,and transarterial *** treatments can lead to changes on imaging that make determination of residual/recurrent disease *** literature-based review dis-cusses the expected postimaging findings following LRT.

关键词： Hepatocellular carcinoma(HCC) Transarterial chemoembolization(TACe) Thermal ablation Transarterial radioembolization(TARe) Magnetic resonance imaging(MRI)

The role of the N-methyl-D-aspartate receptor in the proliferation of adult hippocampal neural stem and precursor cells

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Science China(Life Sciences) 2014年第4期57卷 403-411页

作者： taylor Chanel j He RongQiao BARTLeTT Perry F Queensland Brain Institute The University of QueenslandBrisbane Institute of Biophysics Chinese Academy of Sciences

New neurons are continuously generated from resident pools of neural stem and precursor cells(NSPCs)in the adult *** are multiple pathways through which adult neurogenesis is regulated,and here we review the role of the N-methyl-D-aspartate receptor(NMDAR)in regulating the proliferation of NSPCs in the adult ***-dependent learning tasks,enriched environments,running,and activity-dependent synaptic plasticity,all potently up-regulate hippocampal NSPC *** first consider the requirement of the NMDAR in activity-dependent synaptic plasticity,and the role the induction of synaptic plasticity has in regulating NSPCs and newborn *** address how specific NMDAR agonists and antagonists modulate proliferation,both in vivo and in vitro,and then review the evidence supporting the hypothesis that NMDARs are present on *** believe it is important to understand the mechanisms underlying the activation of adult neurogenesis,given the potential that endogenous stem cell populations have for repopulating the hippocampus with functional new *** conditions such as age-related memory decline,neurodegeneration and psychiatric disease,mature neurons are lost or become defective;as such,stimulating adult neurogenesis may provide a therapeutic strategy to overcome these conditions.

关键词： adult neurogenesis stem cell precursor cell NMDA receptor hippocampus synaptic plasticity long-term potentiation(LTP)

Strategies towards statistically robust interpretations of in situ U-Pb zircon geochronology

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Geoscience Frontiers 2016年第4期7卷 581-589页

作者： Christopher j.Spencer Christopher L.Kirkland Richard j.M.taylor The Institute of Geoscience Research(TIGeR) Department of Applied GeologyWestern Australian School of MinesCurtin UniversityWA 6102Australia Centre for exploration Targeting-Curtin Node Department of Applied GeologyWestern Australian School of MinesCurtin UniversityWA 6102Australia Australian Research Council Centre of Excellence for Core to Crust Fluid SystemsPerthWA 6102Australia

Zircon U-Pb geochronology has become a keystone tool across earth science, arguably providing the gold standard in resolving deep geological time. The development of rapid in situ analysis of zircon （via laser ablation and secondary ionization mass spectrometry） has allowed for large amounts of data to be generated in a relatively short amount of time and such large volume datasets offer the ability to address a range of geological questions that would otherwise remain intractable （e.g. detrital zircons as a sedi- ment fingerprinting method）. The ease of acquisition, while bringing benefit to the earth science com- munity, has also led to diverse interpretations of geochronological data. In this work we seek to refocus U -Pb zircon geochronology toward best practice by providing a robust statistically coherent workflow. We discuss a range of data filtering approaches and their inherent limitations （e.g. discordance and the reduced chi-squared; MSWD）. We evaluate appropriate mechanisms to calculate the most geologically appropriate age from both 238U/206pb and 207pb/206pb ratios and demonstrate the cross over position when chronometric power swaps between these ratios. As our in situ analytical techniques become progressively more precise, appropriate statistical handing of U-Pb datasets will become increasingly pertinent.

关键词： Zircon Geochronology U-Pb Geostatistics MSWD

Keeping an ear out:size relationship of the tympanic bullae and pinnae in bandicoots and bilbies(Marsupialia:Peramelemorphia)

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Current Zoology 2022年第3期68卷 251-264页

作者： Melissa C.taylor Kenny j.TRAVOUILLON Margaret e.ANDReW Patricia A.FLeMING Natalie M.WARBURTON Terrestrial ecosystem Science and Sustainability Harry Butler InstituteMurdoch University90 South StMurdochWA 6150Australia Collections and Research Western Australian MuseumWelshpoolWA 6106Australia

Bandicoots and bilbies(Order Peramelemorphia)occupy a broad range of habitats across Australia and New Guinea,from open,arid deserts to dense *** once diverse group has been particularly vulnerable to habitat loss and introduced eutherian predators,and numerous species extinctions and range retractions have *** reasons for this loss requires greater understanding of their *** of the pinnae and tympanic bullae varies markedly among *** hearing is important for both predator avoidance and prey location,the variability in ear morphology could reflect specialization and adaptation to specific environments,and therefore be of conservation *** measured 798 museum specimens representing 29 species of *** for phylogenetic relatedness and head length,pinna surface area was weakly negatively correlated with average precipitation(rainfall being our surrogate measure of vegetation productivity/complexity),and there were no environmental correlates with effective diameter(pinna width).Controlling for phylogenetic relatedness and skull length,tympanic bulla volume was negatively correlated with *** that inhabited drier habitats,which would be open and allow sound to carry further with less obstruction,had relatively larger pinnae and tympanic *** contrast,species from higher rainfall habitats,where sounds would be attenuated and diffused by dense vegetation,had the smallest pinnae and bullae,suggesting that low-frequency hearing is not as important in these *** with temperature did not reach statistical *** findings highlight linkages between hearing traits and habitat that can inform conservation and management strategies for threatened species.

关键词： external ear hearing ecology marsupial middle ear sound perception

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The Moon

Acta Geochimica 2016年第1期35卷 1-13页

作者： ross taylor australian national university CanberraAustralia

Recent geochemical and geophysical data from the Moon enable a revision of earlier interpretations regarding lunar origin, structure and bulk composition. earth and Moon show many similarities among their isotopic compositions, but they have evolved in totally dissimilar ways, probably related to the deficiency of water and volatile elements in the Moon as well as the vast differences in size and internal pressure. Some global geochemical differences from the earth such as volatile depletion based on K/U ratios have been established. However, all current lunar samples come from differentiated regions, making the establishment of a bulk composition more reliant on bulk geophysical properties or isotopic similarities; it remains unclear how the latter arose or relate to whole Moon composition. The lack of fractionation effects among the refractory and super-refractory elements indicates that the proto-lunar material seems unlikely to have been vaporized while the presence of volatile elements may place lower limits on proto-lunar temperatures. The apparent lack of geochemical evidence of an impacting body enables other possible impactors, such as comets, to be considered. Although the origin of the Moon remains currently unknown, it is generally believed that the Moon originated as the result of a giant impact on the earth.

关键词： Lunar origin Grand tack model Apollo Volatite elements

enhancing perianal disease management with integrated physical and psychological approaches

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World journal of Clinical Cases 2025年第2期13卷 59-63页

作者： Uchenna e Okpete Haewon Byeon Department of Digital Anti-aging Healthcare(BK21) Inje UniversityGimhae 50834South Korea

This article provides a comprehensive analysis of the study by Hou et al,focusing on the complex interplay between psychological and physical factors in the postoperative recovery(POR)of patients with perianal *** study sheds light on how illness perception,anxiety,and depression significantly influence recovery *** et al developed a predictive model that demonstrated high accuracy in identifying patients at risk of poor *** article explores the critical role of pre-operative psychological assessment,highlighting the need for mental health support and personalized recovery plans in enhancing POR quality.A multidisciplinary approach,integrating mental health professionals with surgeons,anesthesiologists,and other specialists,is emphasized to ensure comprehensive care for *** study’s findings serve as a call to integrate psychological care into surgical practice to optimize outcomes for patients with perianal diseases.

关键词： Perianal disease Post-operative recovery Anxiety Depression Pain management emotional well-being Multidisciplinary approach

Women in visual neural regeneration research

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Neural Regeneration Research 2025年第2期20卷 489-490页

作者： Tonia S.Rex David j.Calkins Vanderbilt eye Institute Vanderbilt University Medical CenterNashvilleTNUSA

The year 2024 marks the 60^(th)anniversary of Title IX and 25 years since the New York Times revealed bias against female faculty members at the Massachusetts Institute of *** take an opportunity here to examine the state of gender bias in a relatively new yet already prominent field,neural regeneration in the visual system,for which there is a well-defined context useful for this *** National eye Institute(NeI)provided the first round of research funding for its Audacious Goals Initiative(AGI)on visual neural regeneration in 2013 and the last round in ***,we focus on this *** sources included PubMed,the National Science Foundation(NSF),the NeI,the Blue Ridge Institute for Medical Research and data from the major professional organization for eye and vision research,the Association for Research in Vision and Ophthalmology(ARVO).

关键词： neural visual Times