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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

Exponential Continuous Non-Parametric Neural Identifier With Predefined Convergence Velocity

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IEEE/CAA journal of Automatica Sinica 2022年第6期9卷 1049-1060页

作者： Mariana ballesteros Rita Q.Fuentes-Aguilar Isaac Chairez Instituto Politécnico Nacional Centro de Innovación y Desarrollo Tecnológico en Cómputo Unidad Profesional Interdisciplinaria de Biotecnología 07340Mexico CityMexico Tecnológico de Monterrey School of Engineering and SciencesZapopanJalisco 45138Mexico Department of Bioprocesses UPIBIInstituto Politécnico Nacional07340Mexico City

This paper addresses the design of an exponential function-based learning law for artificial neural networks(ANNs)with continuous *** ANN structure is used to obtain a non-parametric model of systems with uncertainties,which are described by a set of nonlinear ordinary differential *** novel adaptive algorithms with predefined exponential convergence rate adjust the weights of the *** first algorithm includes an adaptive gain depending on the identification error which accelerated the convergence of the weights and promotes a faster convergence between the states of the uncertain system and the trajectories of the neural *** second approach uses a time-dependent sigmoidal gain that forces the convergence of the identification error to an invariant set characterized by an *** generalized volume of this ellipsoid depends on the upper bounds of uncertainties,perturbations and modeling *** application of the invariant ellipsoid method yields to obtain an algorithm to reduce the volume of the convergence region for the identification *** adaptive algorithms are derived from the application of a non-standard exponential dependent function and an associated controlled Lyapunov *** examples demonstrate the improvements enforced by the algorithms introduced in this study by comparing the convergence settings concerning classical schemes with non-exponential continuous learning *** proposed identifiers overcome the results of the classical identifier achieving a faster convergence to an invariant set of smaller dimensions.

关键词： Exponential Lyapunov functions learning laws non-parametric identifier predefined convergence rate

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

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 major 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

Output Second-order Sliding-mode Control for a Gecko Biomimetic Climbing Robot

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journal of Bionic Engineering 2019年第4期16卷 633-646页

作者： David Cruz-Ortiz Mariana ballesteros-Escamilla Isaac Chairez Alberto Luviano SEPl UPIITAInstitute Politecnico NationalAv.Institute Politecnico National 258007340Mexico Bioprocesses Department UPIBIInstitute Politecnico NationalAv.Acueducto 55007340Mexico

The aim of this study is to propose the output controller to solve the execution of the gait sequence for Gecko Bio-inspired Robotic Devices (GBRDs).The Twisting Controller (TC) serves as basis of the tracking trajectory designed *** TC uses both the tracking error and its estimated derivative,which is calculated by a set of distributed Super-Twisting Algorithms (STAs).Each STA is implemented as a robust and exact *** output-based controller structure corresponds to a sort of decentralized form for robotic ***,each articulation is controlled by an independent TC.A set of proposed references trajectories reproduce the gait cycle of a Real Gecko-Lizard (RG-L).The reference trajectories are proposed as the superposition of sigmoid functions ful-filling the conditions of the Bezzier *** simulations evaluate the GBRD movement enforced by the suggested controller in the horizontal as well as the vertical *** own 3D printed GBRD is the experimental platform aimed to test the distributed *** controlled vacuum pumps are used to adhere the GBRD to the wall surfaces.A set of experimental validations confirm the robustness and the reliability of the proposed controller when its performance is compared with classical output feedback controllers.

关键词： gecko robot biomimetic output-based controller twisting controller Super-Twisting Algorithm(STA)

Constraints on what controls millennial-scale basin-wide denudation across mountain belts, Asia

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Science China Earth Sciences 2025年第1期68卷 74-93页

作者： Ye YANG Steven A.BINNIE Sheng XU Cong-Qiang LIU Tibor j.DUNAI Institute of Surface-Earth System Science School of Earth System Science Tianjin University Institute of Geology and Mineralogy University of Cologne

The terrestrial cosmogenic nuclide dataset consisting of 123310Be measurements of fluvial sediment samples across the Asian mountain belts is used to constrain the influence of climate,tectonics and topography on Earth-surface denudation processes.10Be-derived basin-wide denudation rates were recalculated using a Monte Carlo simulation approach that relied on pixel-by-pixel production *** rates derived span four orders of magnitude,from 7.4±0.7 mm kyr-1（1σ;Central Tibetan Plateau） to 9,646744+777mm kyr-1（1σ;Southern Tibetan Plateau）,with a median of 186 mm *** our results with the traditional basin-wide production rate scaling model using a centroid coordinate and mean elevation,suggests that 96.6%of production rates will be underestimated using the latter method,especially for basins with a large *** bias between both methods reaches as large as 40%,caused by basin hypsometry and the nonlinear scaling of production rate with elevation and *** of the correlations between uniformly recalculated denudation rates and tectonics,climate,topography and rock lithology will facilitate the extrapolation of denudation rates in unknown basins using available *** the perspective of local scales,precipitation coupled with neotectonics-driven landslides is likely the most influential factor in subtropical Taiwan Island,while topography primarily constrains surface denudation rates in the eastern Tibetan Plateau within a narrow range of *** highland regions with glacier coverage,such as the Pamir,central and southern Tibetan Plateau high denudation rates are affected by glaciation,whereas lower precipitation and high erosion base level may limit river incision and evolving relief in the Central Tibetan *** contrast to local-scale findings highlighting a dominant factor constraining denudation rate,our large-scale quantitative analyses across the Asian mountain belts finds that （1） topographic metrics including

关键词： 10Be-derived denudation rate Climate change Tectonic uplift Topographic gradient Asian mountains

Neuroprotection by resveratrol-glucuronide and quercetin-glucuronide via binding to polyphenol-and glycosaminoglycan-binding sites in the laminin receptor

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Neural Regeneration Research 2025年第3期20卷 819-820页

作者： Rayudu Gopalakrishna jennifer Aguilar Emily Lee William j.Mack Department of Integrative Anatomical Sciences Keck School of MedicineUniversity of Southern CaliforniaLos AngelesCAUSA Department of Neurological Surgery Keck School of MedicineUniversity of Southern CaliforniaLos AngelesCAUSA

The dietary polyphenolic compounds resveratrol and quercetin prevent neurodegenerative diseases in experimental models;however, they reach the brain only in nanomolar concentrations in the glucuronidated and sulfated ... 详细信息

关键词： resveratrol molar dated