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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

High mobility group box 1 in the central nervous system:regeneration hidden beneath inflammation

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Neural Regeneration Research 2025年第1期20卷 107-115页

作者： Hanki Kim Bum Jun Kim Seungyon Koh Hyo Jin Cho Xuelian Jin Byung Gon Kim Jun Young Choi Department of Brain science Ajou University School of MedicineSuwonSouth Korea Department of Biomedical sciences Ajou University Graduate School of MedicineSuwonSouth Korea Department of Neurology Ajou University School of MedicineSuwonSouth Korea Department of Geriatrics The Affiliated Suqian First People’s Hospital of Nanjing Medical UniversitySuqianJiangsu ProvinceChina

High-mobility group box 1 was first discovered in the calf thymus as a DNA-binding nuclear protein and has been widely studied in diverse fields,including neurology and ***-mobility group box 1 in the extracellular space functions as a pro-inflammatory damage-associated molecular pattern,which has been proven to play an important role in a wide variety of central nervous system disorders such as ischemic stroke,Alzheimer’s disease,frontotemporal dementia,Parkinson’s disease,multiple sclerosis,epilepsy,and traumatic brain *** drugs that inhibit high-mobility group box 1 as a damage-associated molecular pattern,such as glycyrrhizin,ethyl pyruvate,and neutralizing anti-high-mobility group box 1 antibodies,are commonly used to target high-mobility group box 1 activity in central nervous system *** it is commonly known for its detrimental inflammatory effect,high-mobility group box 1 has also been shown to have beneficial pro-regenerative roles in central nervous system *** this narrative review,we provide a brief summary of the history of high-mobility group box 1 research and its characterization as a damage-associated molecular pattern,its downstream receptors,and intracellular signaling pathways,how high-mobility group box 1 exerts the repair-favoring roles in general and in the central nervous system,and clues on how to differentiate the pro-regenerative from the pro-inflammatory *** targeting high-mobility group box 1 in the central nervous system may benefit from differentiating between the two functions rather than overall suppression of high-mobility group box 1.

关键词： central nervous system damage-associated molecular pattern ethyl pyruvate glycyrhizzin high mobility group box 1 inflammation neural stem cells neurodevelopment oligodendrocyte progenitor cells redox status regeneration

Sonochemical synthesis and characterization of uniform lanthanide orthophosphate(LnPO_4, Ln=La and Ce) nanorods

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Rare Metals 2015年第5期34卷 301-307页

作者： Anukorn Phuruangrat Titipun Thongtem Somchai Thongtem Department of Materials science and Technology Faculty of Science Prince of Songkla University Department of Chemistry Faculty of Science Chiang Mai University Department of Physics and Materials science Faculty of Science Chiang Mai University

Uniform lanthanide orthophosphate （LnPO4, Ln = La and Ce） nanorods were successfully synthesized by a simple ultrasonic irradiation method using lanthanide nitrate salt （Ln（NO3）3-6H2O, Ln = La and Ce） and sodium phosphate （Na3PO4） in aqueous solutions with the pH of 1-3. The products were characterized by X-ray diffraction （XRD）, scanning electron microscopy （SEM）, transmission electron microscopy （TEM）, high-resolution transmission electron microscopy （HRTEM）, Fourier transform infrared spectrometer （FT-IR）, and UV-visible （UV-Vis） spectroscopy. In this research, the products are nanorods of monoclinic LaPO4 and hexagonal CePOa structures and the vibration modes of PO4^3-, including the strong peaks at 227 nm for LaPO4, and at 225 and 278 nm for CePO4.

关键词： Ultrasonic Lanthanide orthophosphate Nanorods irradiation

Major Development Under Gaussian Filtering Since Unscented Kalman Filter

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IEEE/CAA Journal of Automatica Sinica 2020年第5期7卷 1308-1325页

作者： Abhinoy Kumar Singh IEEE Department of Electrical Engineering Indian Institute of Technology Indore

Filtering is a recursive estimation of hidden states of a dynamic system from noisy *** problems appear in several branches of science and technology,ranging from target tracking to biomedical monitoring.A commonly practiced approach of filtering with nonlinear systems is Gaussian *** early Gaussian filters used a derivative-based implementation,and suffered from several drawbacks,such as the smoothness requirements of system models and poor stability.A derivative-free numerical approximation-based Gaussian filter,named the unscented Kalman filter(UKF),was introduced in the nineties,which offered several advantages over the derivativebased Gaussian *** the proposition of UKF,derivativefree Gaussian filtering has been a highly active research *** paper reviews significant developments made under Gaussian filtering since the proposition of *** review is particularly focused on three categories of developments:i)advancing the numerical approximation methods;ii)modifying the conventional Gaussian approach to further improve the filtering performance;and iii)constrained filtering to address the problem of discrete-time formulation of process *** review highlights the computational aspect of recent developments in all three *** performance of various filters are analyzed by simulating them with real-life target tracking problems.

关键词： Bayesian framework cubature rule-based filtering Gaussian filters Gaussian sum and square-root filtering nonlinear filtering quadrature rule-based filtering unscented transformation

Spin-correlation function of the fully frustrated Ising model and ±J Ising spin glass on a square lattice

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Chinese Physics B 2013年第6期22卷 549-552页

作者： M Y Ali J Poulter Department of Mathematics Faculty of ScienceMahidol University

In this work we study the correlation function of the ground state of a two-dimensional fully frustrated Ising model as well as spin glass. The Pfaffian method is used to calculate free energy and entropy as well as the correlation function. We estimate the exponent of spin correlation function for the fully frustrated model and spin glass. In this paper an overview of the latest results on the spin correlation function is presented.

关键词： spin glass correlation function exponent square lattice

Fractionally Delayed Kalman Filter

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IEEE/CAA Journal of Automatica Sinica 2020年第1期7卷 169-177页

作者： Abhinoy Kumar Singh Discipline of Electrical Engineering Indian Institute of Technology Indore

The conventional Kalman filter is based on the assumption of non-delayed measurements. Several modifications appear to address this problem, but they are constrained by two crucial assumptions: 1) the delay is an integer multiple of the sampling interval, and 2) a stochastic model representing the relationship between delayed measurements and a sequence of possible non-delayed measurements is known. Practical problems often fail to satisfy these assumptions, leading to poor estimation accuracy and frequent track-failure. This paper introduces a new variant of the Kalman filter, which is free from the stochastic model requirement and addresses the problem of fractional *** proposed algorithm fixes the maximum delay(problem specific), which can be tuned by the practitioners for varying delay possibilities. A sequence of hypothetically defined intermediate instants characterizes fractional delays while maximum likelihood based delay identification could preclude the stochastic model requirement. Fractional delay realization could help in improving estimation accuracy. Moreover, precluding the need of a stochastic model could enhance the practical applicability. A comparative analysis with ordinary Kalman filter shows the high estimation accuracy of the proposed method in the presence of delay.

关键词： Gaussian likelihood Kalman filter optimal Kalman gain randomly delayed measurements

The use of hydrogeochemical analyses and multivariate statistics for the characterization of thermal springs in the Constantine area, Northeastern Algeria

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Acta Geochimica 2019年第2期38卷 292-306页

作者： Riad Kouadra Abdeslam Demdoum Nabil Chabour Rebiha Benchikh Department of Geological sciences University of Frères Mentouri (Constantine 1) Department of Earth sciences University of Setif 1

This paper deals with the results of a hydrogeochemistry study on the thermal waters of the Constantine area, Northeastern Algeria, using geochemical and statistical tools. The samples were collected in December2016 from twelve hot springs and were analyzed for physicochemical parameters(electric conductivity, p H,total dissolved solids, temperature, Ca, Mg, Na, K, HCO_3,Cl, SO_4, and SiO_2). The waters of the thermal springs have temperatures varying from 28 to 51 °C and electric conductivity values ranging from 853 to 5630 l S/cm. Q-mode Cluster analysis resulted in the determination of two major water types: a Ca–HCO_3–SO_4 type with a moderate salinity and a Na–K–Cl type with high salinity. The plot of the major ions versus the saturation indices suggested that the hydrogeochemistry of thermal groundwater is mainly controlled by dissolution/precipitation of carbonate minerals, dissolution of evaporite minerals(halite and gypsum), and ion exchange of Ca(and/or Mg) by Na. The Gibbs diagram shows that evaporation is another factor playing a minor role. Principal Component Analysis produced three significant factors which have 88.2% of totalvariance that illustrate the main processes controlling the chemistry of groundwaters, which are respectively: the dissolution of evaporite minerals(halite and gypsum), ion exchange, and dissolution/precipitation of carbonate minerals. The subsurface reservoir temperatures were calculated using different cation and silica geothermometers and gave temperatures ranging between 17 and 279 °C. The Na–K and Na–K-Ca geothermometers provided high temperatures(up to 279 °C), whereas, estimated geotemperatures from K/Mg geothermometers were the lowest(17–53 °C). Silica geothermometers gave the most reasonable temperature estimate of the subsurface waters overlap between 20 and 58 °C, which indicate possible mixing with cooler Mg groundwaters indicated by the Na–K–Mg plot in the immature water field and in silica and chloride mixing models

关键词： Hydrogeochemistry Thermal waters-Multivariate statistical analysis Silica geothermometers Mixing models Cold groundwaters

Weathering and accumulation of trace elements in the soils of the Porali Plain, Balochistan: repercussions in agriculture

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Acta Geochimica 2024年第2期43卷 214-234页

作者： Maria Kaleem Erum Bashir Shahid Naseem Tahir Rafique Bushra Shahab Department of Geology University of KarachiKarachi 75270Pakistan Applied Chemistry Research Centre PCSIR Laboratories ComplexKarachi 75280Pakistan

This study is theﬁrst attempt to assess the nature of the soil,especially on the western side of the Porali Plain in Balochistan;a new emerging agriculture hub,using weathering and pollution indices supplemented by multi-variate analysis based on geochemical *** outcomes of this study are expected to help farmers in soil manage-ment and selecting suitable crops for the ***-ﬁve soil samples were collected,mainly from the arable land of the Porali *** drying and coning-quarter-ing,soil samples were analyzed for major and trace ele-ments using the XRF technique;sieving and hydrometric methods were employed for granulometric ***-mated data were analyzed using Excel,SPSS,and Surfer software to calculate various indices,correlation matrix,and spatial *** granulometric analysis showed that 76%of the samples belonged to loam types of soil,12%to sand type,and 8%to silt *** indices:CIA,CIW,PIA,PWI,WIP,CIX,and ICV were calculated to infer the level of *** indices reﬂect mod-erate to intense weathering;supported by K_(2)O/AI_(2)O_(3),Rb/K_(2)O,Rb/Ti,and Rb/Sr *** of the geo-ac-cumulation and Nemerow Pollution indices pinpoint rela-tively high concentrations of Pb,Ni,and Cr concentration in the *** correlation matrix and Principal Compo-nent Analysis show that the soil in this study area is mainly derived from the weathering of igneous rocks of Bela Ophiolite(Cretaceous age)and Jurassic sedimentary rocks of Mor Range having SEDEX/MVT type *** may result in the undesirable accumulation of certain trace elements which adversely affects crops.

关键词： Weathering indices Pollution indices Accumulation Repercussions Trace elements Multivariate analyses Porali Plain Balochistan

Triton X-100 induced cuboid-like BiVO4 microsphere with high photocatalytic performance

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Journal of Environmental sciences 2017年第5期29卷 257-265页

作者： Cheewita Suwanchawalit Supat Buddee Sumpun Wongnawa Department of Chemistry Faculty of Science Silpakorn University Sahara Chandra Palace Campus Nakornpathom 73000 Thailand Department of General science Faculty of Education Nakhon Si Thammarat Rajabhat University Nakhon Si Thammarat 80280 Thailand Department of Chemistry Faculty of Science Prince of Songkla University Hat Yai Songkhla 90112 Thailand

A highly active visible light-induced BiVO4 photocatalyst was prepared by a simple co-precipitation method using ammonia solution as a pH adjustor and Triton X-100（TX100） as a structure directing *** physical properties of the prepared BiVO4photocatalyst were investigated by several techniques such as X-ray diffractometer（XRD）,Brunauer-Emmett-Teller（BET） surface area measurement,scanning electron microscopy（SEM）,energy dispersive analysis（EDS）,Fourier-transformed infrared spectroscopy（FT-IR）,photoluminescence（PL）,and UV-Vis diffused reflectance spectroscopy（DRS）.XRD results revealed the existence of a monoclinic scheelite structure in both the unmodified and the modified *** TX100 played a crucial role to control the cuboid-like shape morphology of *** DRS results showed that the as-prepared cuboid BiVO4 possessed enhanced visible light absorption range compared with that of the unmodified *** photocatalytic activity was evaluated via indigo carmine（IC） degradation under visible light *** modified BiVO4 showed higher photocatalytic efficiency than the unmodified BiVO4 and the commercial P25-TiO2 which could be ascribed to the better charge separation efficiency.

关键词： Bismuth vanadate Triton X100 Co-precipitation Indigo carmine Dye decolofization