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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

Direct Numerical Simulation of Convective Heat Transfer in a Zero-Pressure-Gradient Boundary Layer with Supercritical Water

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journal of Thermal Science 2012年第1期21卷 49-59页

作者： C. Azih j.r. Brinkerhoff M.I. Yaras Department of Mechanical and Aerospace Engineering Carleton University

Experimental research has long shown that forced-convective heat transfer in wall-bounded turbulent flows of fluids in the supercritical thermodynamic state is not accurately predicted by correlations that have been developed for single-phase fluids in the subcritical thermodynamic state. In the present computational study, the statistical properties of turbulent flow as well as the development of coherent flow structures in a zero-pressuregradient flat-plate boundary layer are investigated in the absence of body forces, where the working fluid is in the supercritical thermodynamic state. The simulated boundary layers are developed to a friction reynolds number of 250 for two heat-flux to mass-flux ratios corresponding to cases where normal heat transfer and improved heat transfer are observed. In the case where improved heat transfer is observed, spanwise spacing of the near-wall coherent flow structures is reduced due to a relatively less stable flow environment resulting from the lower magnitudes of the wall-normal viscosity-gradient profile.

关键词： boundary layer turbulence supercritical fluids improved heat transfer DNS

Moore-Gibson-Thompson theory for thermoelastic dielectrics

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Applied Mathematics and Mechanics(English Edition) 2021年第2期42卷 309-316页

作者： j.r.FErNANDEZ r.QUINTANILLA Department of Applied Mathematics I University of VigoCampus As Lagoas MarcosendeVigo 36310Spain Department of Mathematics Polytechnic University of CataloniaTerrassa 08222Spain

We consider the system of equations determining the linear thermoelastic deformations of dielectrics within the recently called Moore-Gibson-Thompson(MGT)***,we obtain the system of equations for such a ***,we consider the case of a rigid solid and show the existence and the exponential decay of ***,we consider the thermoelastic case and obtain the existence and the stability of the *** decay of solutions in the one-dimensional case is also recalled.

关键词： Moore-Gibson-Thompson(MGT)thermoelastic dielectrics existence semigroup exponential decay

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.r.bson 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 inj.r.(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

NUMErICAL INVESTIGATION ON FLOW CHArACTErISTICS IN rIBBED BEND

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journal of Hydrodynamics 2001年第2期13卷 111-120页

作者： Yao, j. Fan, j.r. Zhang, X.Y. Cen, K.F. Dept. of Energy Engineering Zhejiang Univ. Hangzhou 310027 China

Previous research by the author has proved that ribbed bend technology is a simple and efficient anti-erosion method. The present paper is a further study to unveil the mechanism of the technology by using numerical method. The flow characteristics in ribbed bend were studied. A k-Ε turbulence model was used and the simulations were carried out in the body-fitted coordinates. This procedure was confirmed to be credible by showing the satisfactory agreement between the predications and experimental results. It is concluded that the character of the longitudinal flow in ribbed bend especially in the concave parts between ribs has a beneficial effect on increasing the anti-erosion ability of ribbed bend but the secondary flow has little effect on determining the particle trajectory.

关键词： Boundary conditions Differential equations Erosion Mathematical models Numerical methods Three dimensional

AN ECO- AND INNO-PrODUCT DESIGN SYSTEM APPLYING INTEGrATED AND INTELLIGENT QFDE AND TrIZ METHODOLOGY

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journal of Systems Science and Systems Engineering 2011年第4期20卷 443-459页

作者： Amy j.C. TrAPPEY j.r.y j.r. OU Gilbert Y.P. LIN Meng-Yu CHEN Department of Industrial Engineering and Engineering Management National Tsing Hua University Taiwan China Department of Industrial Engineering and Management National Taipei University of Technology Taiwan China Department of Business Administration Southern Taiwan University Taiwan China

The green environmental laws and regulations are legislated, implemented, and enforced in many countries and economic regions. The provision of green products and services are the fast growing trend in global consumer markets. Therefore, introducing new products with environmental considerations becomes critical for global brand manufacturers. This research depicts an integrated and intelligent eco- and inno-product design methodology to support environmental friendly green product development. The methodology adopts approaches, such as life cycle assessment （LCA）, quality function deploymnet for environement （QFDE）, theory of inventive problem solving （TrIZ） and back-propagation network （BPN） to achieve eco- and inno-design objectives. LCA evaluates and compares the environmental impacts of production. QFDE transforms high-level concerns of environment into design requirements. When there are many historical QFDE data, the BPN prediction model is trained and deployed to automate the specifications of green design improvement. TrIZ is to support the creation of innovative product design ideas effectively and efficiently during the concept design stage. Finally, this paper presents two eco-design cases of power adaptor to demonstrate the proposed methodology.

South Asian dairy smallholders: A scoping review of practices and zoonoses

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Asian Pacific journal of Tropical Medicine 2023年第10期16卷 446-452页

作者： Emily Schembri Angus j D Campbell juan Pablo villanueva-Cabezas Melbourne School of Population and Global Health The University of MelbourneVictoriaAustralia The Nossal Institute for Global Health The University of MelbourneVictoriaAustralia The Peter Doherty Institute for Infection and Immunity The University of Melbourne and the Royal Melbourne HospitalVictoriaAustralia

Objective: To identify and discuss on-farm management practices linked to bacterial zoonosis risk in smallholder dairy farmers in South Asia. Methods: This scoping review was conducted as per the PrISMA-Scr guidelines. Five hundred and two publications were retrieved from five online databases using a comprehensive search strategy. Studies were selected if they discussed a farm management practice which impacted human health within a South Asian country. results: Twenty-two studies were included. Seven management practices relevant to farmers, livestock and their shared environment were identified including raw milk consumption, farm hygiene management, personal protective equipment uses, animal vaccination, cleaning udders, hand washing and disposal of afterbirth materials. Preventive practices were found to be utilized at lower frequencies compared to risk increasing practices. Awareness of bacterial zoonoses is particularly low within the region. Conclusions: Based on the results of this review, it was determined that improving farmer awareness of bacterial zoonotic diseases may favor several of the presented leverage points within the South Asian smallholder dairy system. relying on formal school education to improve this awareness may not solve this problem, instead, more focus on accessible and affordable zoonoses education and farming programs is required.

关键词： Management practices Zoonoses Dairy Smallholder system

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 maj.r.professional organization for eye and vision research,the Association for research in Vision and Ophthalmology(ArVO).

关键词： neural visual Times

Generation of brain vascular heterogeneity:recent advances from the perspective of angiogenesis

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Neural regeneration research 2025年第7期20卷 2013-2014页

作者： Nathanael j.Lee ryota L.Matsuoka Department of Neurosciences Lerner Research InstituteCleveland ClinicClevelandOHUSA Department of Molecular Medicine Cleveland Clinic Lerner College of MedicineCase Western Reserve UniversityClevelandOHUSA

Heterogeneous proper t i es of vascular endothelial cells in the brain:The brain displays large energy dynamics and consumption,and this high level of metabolic demands is fulfilled by a continuous supply of glucose and oxygen through its vascular *** vasculature consists of highly divergent blood vessel branches,giving rise to a dense network of capillaries that supply blood to all cells across the *** elaborated vascular network is thought to develop via angiogenesis,a process in which new blood vessels grow from pre-existing *** capillaries exhibit organotypic features distinct from other tissues and are formed primarily by two maj.r.endothelial cell(EC)types:those that form the semi-permeable blood-brain barrier(BBB)and those that develop highly permeable pores known as fenestrae(Matsuoka et al.,2022).The structural and functional differences between BBB and fenestrated vascular ECs represent a fundamental feature of brain vasculature and form the foundation for both brain function and homeostasis.

关键词： angiogenesis homeostasis network