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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

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用于再循环水产养殖的太阳能加热系统

渔业现代化 2007年第6期34卷 38-38页

作者： r.j.fuller

为减少小型再循环养殖系统为维持水温而对常规能源的需求,人们对使用被动式和主动式太阳能技术始终抱有兴趣.本文描述了一个仿真模型的使用,该模型可预测一个置于双层聚乙烯温室中的10.6 m3的再循环养殖系统(rAS)在两种不同气候状态下... 详细信息

为减少小型再循环养殖系统为维持水温而对常规能源的需求,人们对使用被动式和主动式太阳能技术始终抱有兴趣.本文描述了一个仿真模型的使用,该模型可预测一个置于双层聚乙烯温室中的10.6 m3的再循环养殖系统(rAS)在两种不同气候状态下的年常规能源消耗量.水温维持在22.5℃,每天的循环率是池水的10%;温室组合了简单的无釉太阳能收集器,以进一步减少能源消耗.对增加太阳能收集器的面积对太阳能保证率和有效能利用的影响进行了预测.最后,该模型用于研究温室内罩的冷凝现象,换气次数和能源消耗之间的关系.

关键词：太阳能加热系统水产养殖再循环循环养殖系统太阳能收集器能源消耗量仿真模型太阳能技术

Integrins and their potential roles in mammalian pregnancy

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journal of Animal Science and Biotechnology 2024年第1期15卷 1-19页

作者： Gregory A.johnson robert C.Burghardt fuller W.Bazer Heewon Seo joe W.Cain Department of Veterinary Integrative Biosciences School of Veterinary Medicine and Biomedical SciencesTexas A&M UniversityCollege StationTX 77843‑4459USA Department of Animal Science College of Agriculture and Life SciencesTexas A&M UniversityCollege StationTX 77843‑2471USA

Integrins are a highly complex family of receptors that, when expressed on the surface of cells, can mediate reciprocal cell-to-cell and cell-to-extracellular matrix(ECM) interactions leading to assembly of integrin adhesion complexes(IACs) that initiate many signaling functions both at the membrane and deeper within the cytoplasm to coordinate processes including cell adhesion, migration, proliferation, survival, differentiation, and metabolism. All metazoan organisms possess integrins, and it is generally agreed that integrins were associated with the evolution of multicellularity, being essential for the association of cells with their neighbors and surroundings, during embryonic development and many aspects of cellular and molecular biology. Integrins have important roles in many aspects of embryonic development, normal physiology, and disease processes with a multitude of functions discovered and elucidated for integrins that directly influence many areas of biology and medicine, including mammalian pregnancy, in particular implantation of the blastocyst to the uterine wall, subsequent placentation and conceptus(embryo/fetus and associated placental membranes) development. This review provides a succinct overview of integrin structure, ligand binding, and signaling followed with a concise overview of embryonic development, implantation, and early placentation in pigs, sheep, humans, and mice as an example for rodents. A brief timeline of the initial localization of integrin subunits to the uterine luminal epithelium(LE) and conceptus trophoblast is then presented, followed by sequential summaries of integrin expression and function during gestation in pigs, sheep, humans, and rodents. As appropriate for this journal, summaries of integrin expression and function during gestation in pigs and sheep are in depth, whereas summaries for humans and rodents are brief. Because similar models to those illustrated in Fig. 1, 2, 3, 4, 5 and 6 are present throughout the sc

关键词： Humans Implantation Integrins Pigs Pregnancy rodents Sheep

Mathematical modelling of ball and plate system with experimental and correlation function-based model validation

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Control Theory and Technology 2024年第2期22卷 326-341页

作者： T.r.Dil Kumar S.j.Mija Department of Electrical Engineering National Institute of Technology CalicutKozhikode673601KeralaIndia

The ball and plate system is an inherently nonlinear under actuated benchmark system used for validating the performance of various control schemes. A mathematical model depicting the dynamics close to that of the system is very much required for such a test bed. In this correspondence, the complete nonlinear model, a simplified nonlinear model, and a linearized model of the ball and plate system are developed. The system comprises a ball and plate mechanism and a rotary servo unit. The ball and plate mechanism is modelled using the Euler–Lagrange method, whereas the rotary servo subsystem is modelled from the first principles. The nonlinear model of the combined system is developed by including the dynamics of the servo motor with gear and rolling resistance between the ball and the plate. The simplified nonlinear model of the system is obtained with suitable assumptions. The model is linearized around the operating point using the jacobian. The validity of the developed models is investigated through correlation function analysis. The open-loop response of the three models, viz., nonlinear, simplified nonlinear, and linearized models, is analyzed in the MATLAB/Simulink platform. Since the open-loop system is unstable, the experimental validation of the model is performed with a double-loop PSO (particle swarm optimization) PID control scheme.

关键词： Ball and plate system Correlation function Model validation Particle swarm optimization(PSO) PSO PID controller

Dietary fibre effects and the interplay with exogenous carbohydrases in poultry nutrition

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Animal Nutrition 2024年第1期16卷 231-240页

作者： Michael r.Bedford Birger Svihus Aaron j.Cowieson AB Vista Feed Ingredients Ltd Norwegian University of Life Sciences DSM Nutritional Products

A comprehensive understanding of the role of dietary fibre in non-ruminant animal production is elusive. Equivocal and conflated definitions of fibre coupled with significant analytical complexity,interact with poorly defined host and microbiome relationships. Dietary fibre is known to influence gut development, feed intake and passage rate, nutrient absorption, microbiome taxonomy and function, gut p H, endogenous nutrient loss, environmental sustainability, animal welfare and more. Whilst significant gaps persist in our understanding of fibre in non-ruminant animal production, there is substantial interest in optimizing the fibre fraction of feed to induce high value phenotypes such as improved welfare,live performance and to reduce the environmental footprint of animal production systems. In order to achieve these aspirational goals, it is important to tackle dietary fibre with the same level of scrutiny as is currently done for other critical nutrient classes such as protein, minerals and vitamins. The chemical,mechanical and nutritional role of fibre must be explored at the level of monomeric sugars, oligosaccharides and polysaccharides of varying molecular weight and decoration, and this must be in parallel to standardisation of analytical tools and definitions for speciation. To further complicate subject, exogenous carbohydrases recognise dietary fibre as a focal substrate and have varying capacity to generate lower molecular weight carbohydrates that interact differentially with the host and the enteric microbiome. This short review article will explore the interactive space between dietary fibre and exogenous carbohydrases and will include their nutritional and health effects with emphasis on functional development of the gut, microbiome modulation and host metabolism.

关键词： Fibre Carbohydrase Nutrition Microbiome Gastro-intestinal development Feed

On an isotropic porous solid cylinder:the analytical solution and sensitivity analysis of the pressure

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Applied Mathematics and Mechanics(English Edition) 2024年第9期45卷 1499-1522页

作者： H.ASGHArI L.MILLEr r.PENTA j.MErODIO Departamento de Matematica Aplicada a las TIC ETS de Ingenieria de Sistemas InformaticosUniversidad Politecnica de MadridMadrid 28031Spain School of Mathematics and Statistics University of GlasgowGlasgow G128QQU.K.

Within this work,we perform a sensitivity analysis to determine the influence of the material input parameters on the pressure in an isotropic porous solid *** provide a step-by-step guide to obtain the analytical solution for a porous isotropic elastic cylinder in terms of the pressure,stresses,and elastic *** obtain the solution by performing a Laplace transform on the governing equations,which are those of Biot's poroelasticity in cylindrical polar *** enforce radial boundary conditions and obtain the solution in the Laplace transformed domain before reverting back to the time *** sensitivity analysis is then carried out,considering only the derived pressure *** analysis finds that the time t,Biot's modulus M,and Poisson's ratio ν have the highest influence on the pressure whereas the initial value of pressure P_(0) plays a very little role.

关键词： sensitivity analysis Laplace transform cylindrical polar coordinate Biot's modulus cylinder

Molecular Oxygen Abundance in Galactic Massive Star Formation regions Based on SWAS Observations

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research in Astronomy and Astrophysics 2024年第9期24卷 83-98页

作者： Bing-ru Wang Di Li Paul F.Goldsmith jingwen Wu Chao-Wei Tsai Donghui Quan Xia Zhang junzhi Wang Gary j.Melnick jin-Zeng Li Gary A.fuller jinjin Xie National Astronomical Observatories Chinese Academy of SciencesBeijing 100101China Tsinghua University Beijing 100084China University of Chinese Academy of Sciences Beijing 100049China research Center for Astronomical Computing Zhejiang LaboratoryHangzhou 311100China jet Propulsion Laboratory California Institute of TechnologyPasadenaCA 91109USA Institute for Frontiers in Astronomy and Astrophysics Beijing Normal UniversityBeijing 102206China Xinjiang Astronomical Observatory Chinese Academy of SciencesUrumqi 830011China Guangxi Key Laboratory for relativistic Astrophysics School of Physical Science and TechnologyGuangxi UniversityNanning 530004China Center for Astrophysics Harvard&Smithsonian60 Garden StreetMS 66CambridgeMA 02138USA jodrell Bank Center for Astrophysics Department of Physics&AstronomyUniversity of ManchesterOxford RoadManchesterM139PLUK I.Physikalisches Institut University of CologneZülpicher Str.7750937 KölnGermany

Molecular oxygen abundance is a key parameter in understanding the chemical network of the interstellar *** estimate the molecular oxygen column density and abundance for a sample of Galactic massive star formation regions based on observations from the Submillimiter Wave Astronomy Satellite(SWAS)*** obtained an averaged O_(2)spectrum based on this sample using the(SWAS)survey data(O_(2),487.249 GHz,N=3-1,j=3-2).No emission or absorption feature is seen around the supposed central velocity with a total integration time of t_(total)=8.67×10^(3)hr and an rms noise per channel of 1.45 m *** a kinetic temperature T_(kin)=30 K,we derive the 3σupper limit of the O_(2)column density to be 3.3×10^(15)cm^(-2),close to the lowest values reported in Galactic massive star formation regions in previous *** corresponding O_(2)abundance upper limit is6.7×10^(-8),lower than all previous results based on SWAS observations and is close to the lowest reported value in massive star formation *** a galactic scale,our statistical results confirm a generally low O_(2)abundance for Galactic massive star formation *** abundance is also lower than results reported in extragalactic sources.

关键词： ISM:molecules galaxies:abundances ISM:lines and bands Galaxy:abundances

Stable diffusion gradients in microfluidic conduits bounded by fluid walls

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Microsystems & Nanoengineering 2024年第3期10卷 357-367页

作者： Federico Nebuloni Cyril Deroy Peter r.Cook Edmond j.Walsh Department of Engineering Science Osney Thermo-Fluids InstituteUniversity of OxfordOxford OX20ESUK Sir William Dunn School of Pathology University of OxfordOxford OX13REUK

Assays mimicking in vitro the concentration gradients triggering biological responses like those involved in fighting infections and blood clotting are essential for biomedical *** assays prove especially attractive as they allow precise control of gradient shape allied to a reduction in *** microfluidic devices are fabricated using solid plastics that prevent direct access to responding ***-walled microfluidics allows the manufacture of circuits on standard Petri dishes in seconds,coupled to simple operating methods;cell-culture medium sitting in a standard dish is confined to circuits by fluid walls made of an immiscible *** develop and experimentally validate an analytical model of diffusion between two or more aqueous streams flowing at different rates into a fluid-walled conduit with the cross-section of a circular *** solid walls,fluid walls morph during flows as pressures fall,with wall shape changing down the *** model is validated experimentally for Fourier numbersrescein diffusing between laminar *** enables a priori prediction of concentration gradients throughout a conduit,so allowing rapid circuit design as well as providing bioscientists with an accurate way of predicting local concentrations of bioactive molecules around responsive and nonresponsive cells.

关键词： walls diffusion attractive

Single-atom Pt on carbon nanotubes for selective electrocatalysis

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Carbon Energy 2024年第1期6卷 63-71页

作者： Samuel S.Hardisty Xiaoqian Lin Anthony r.j.Kucernak David Zitoun Department of Chemistry Bar Ilan Institute of Nanotechnology and Advanced Materials(BINA)Bar Ilan UniversityRamat GanIsrael Department of Chemistry Molecular Science Research HubImperial College LondonLondonUK

Utilizing supported single atoms as catalysts presents an opportunity to reduce the usage of critical raw materials such as platinum,which are essential for electrochemical reactions such as hydrogen oxidation reaction(HOr).Herein,we describe the synthesis of a Pt single electrocatalyst inside single-walled carbon nanotubes(SWCNTs)via a redox *** via electron microscopy,X-ray photoelectron microscopy,and X-ray absorption spectroscopy show the single-atom nature of the *** electrochemical behavior of the sample to hydrogen and oxygen was investigated using the advanced floating electrode technique,which minimizes mass transport limitations and gives a thorough insight into the activity of the *** single-atom samples showed higher HOr activity than state-of-the-art 30%Pt/C while almost no oxygen reduction reaction activity in the proton exchange membrane fuel cell operating *** selective activity toward HOr arose as the main fingerprint of the catalyst confinement in the SWCNTs.

关键词： confinement electrocatalysis hydrogen platinum single atom catalysts