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SET结合因子2(SBF2)突变导致CMT4B和青少年型青光眼

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世界核心医学期刊文摘(眼科学分册) 2005年第1期 6-6页

作者：肖颖 hirano R takashima h Umehara F Kag os hima Kag oshima Univ.School of Medicine Dept.of Neurolog y and Ge diatrics 8-35-1Sakurag aoka Japan.Dr. 890-8520 Kag oshima city

The authors report a Japanese family segregating autosomal recessive Charcot-Marie-Tooth dis ease(CMT )with focally folded myelin,juvenile-onset glau coma,and a nonsense mutation of SET binding factor 2(SBF2).The consistent phenotypic features associated with SBF2mutations are early-onset demyelinating neuropathy,myelin folding,and markedly decreased motor nerve conduction velocities;glaucoma associates with SBF2nonse nse mutations.

关键词：结合因子青少年型青光眼 SBF2 CMT4B SET

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吸烟对冠状动脉斑块组成的影响

临床荟萃 2014年第10期29卷 1164-1164页

作者： Kumagai S Amano T takashima h 武峪峰

吸烟与动脉粥样硬化相关，并且是心血管疾病的一个重要危险因素。该研究采用背向散射血管内超声（IB-IVUS）评估了吸烟对冠状动脉斑块成分的影响。选择连续143例接受经皮冠状动脉介入治疗的患者，通过采访获得疾病史、以及吸烟习惯。要... 详细信息

吸烟与动脉粥样硬化相关，并且是心血管疾病的一个重要危险因素。该研究采用背向散射血管内超声（IB-IVUS）评估了吸烟对冠状动脉斑块成分的影响。选择连续143例接受经皮冠状动脉介入治疗的患者，通过采访获得疾病史、以及吸烟习惯。要求参与者报告目前是否是吸烟、戒烟或从未吸过烟。根据访谈结果，将患者分为以下3组：目前吸烟、以前吸烟和从不吸烟者。对于患者进行了传统和IB-IVUS组织特性分析。

关键词：冠状动脉斑块吸烟习惯经皮冠状动脉介入治疗组成动脉粥样硬化心血管疾病血管内超声危险因素

Skeletal muscle as a molecular and cellular biomarker of disease progression in amyotrophic lateral sclerosis:a narrative review

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Neural Regeneration Research 2024年第4期19卷 747-753页

作者： Peter h.King Department of Neurology and Center for Neurodegeneration and Experimental Therapeutics The University of Alabama at BirminghamBirminghamALUSA Birmingham Veterans Affairs Medical Center BirminghamALUSA

Amyotrophic lateral sclerosis is a fatal multisystemic neurodegenerative disease with motor neurons being a primary target.Although progressive weakness is a hallmark feature of amyotrophic lateral sclerosis,there is considerable heterogeneity,including clinical presentation,progression,and the underlying triggers for disease initiation.Based on longitudinal studies with families harboring amyotrophic lateral sclerosis-associated gene mutations,it has become apparent that overt disease is preceded by a prodromal phase,possibly in years,where compensatory mechanisms delay symptom onset.Since 85-90%of amyotrophic lateral sclerosis is sporadic,there is a strong need for identifying biomarkers that can detect this prodromal phase as motor neurons have limited capacity for regeneration.Current Food and Drug Administration-approved therapies work by slowing the degenerative process and are most effective early in the disease.Skeletal muscle,including the neuromuscular junction,manifests abnormalities at the earliest stages of the disease,before motor neuron loss,making it a promising source for identifying biomarkers of the prodromal phase.The accessibility of muscle through biopsy provides a lens into the distal motor system at earlier stages and in real time.The advent of“omics”technology has led to the identification of numerous dysregulated molecules in amyotrophic lateral sclerosis muscle,ranging from coding and non-coding RNAs to proteins and metabolites.This technology has opened the door for identifying biomarkers of disease activity and providing insight into disease mechanisms.A major challenge is correlating the myriad of dysregulated molecules with clinical or histological progression and understanding their relevance to presymptomatic phases of disease.There are two major goals of this review.The first is to summarize some of the biomarkers identified in human amyotrophic lateral sclerosis muscle that have a clinicopathological correlation with disease activity,evidence of a similar dysregulation in the SO

关键词： amyotrophic lateral sclerosis biomarkers clinicopathological correlation disease progression muscle biomarkers neurogenic atrophy neuromuscular junction non-coding RNAs presymptomatic stages skeletal muscle SOD1G93A mouse model

Free vibration and buckling analysis of polymeric composite beams reinforced by functionally graded bamboo fbers

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Applied Mathematics and Mechanics(English Edition) 2024年第3期45卷 543-562页

作者： h.M.FEIZABAD M.h.YAS Department of Mechanical Engineering Razi UniversityKermanshah ***Iran

Natural fibers have been extensively researched as reinforcement materials in polymers on account of their environmental and economic advantages in comparison with synthetic fibers in the recent years.Bamboo fibers are renowned for their good mechanical properties,abundance,and short cycle growth.As beams are one of the fundamental structural components and are susceptible to mechanical loads in engineering applications,this paper performs a study on the free vibration and buckling responses of bamboo fiber reinforced composite(BFRC)beams on the elastic foundation.Three different functionally graded(FG)layouts and a uniform one are the considered distributions for unidirectional long bamboo fibers across the thickness.The elastic properties of the composite are determined with the law of mixture.Employing hamilton’s principle,the governing equations of motion are obtained.The generalized differential quadrature method(GDQM)is then applied to the equations to obtain the results.The achieved outcomes exhibit that the natural frequency and buckling load values vary as the fiber volume fractions and distributions,elastic foundation stiffness values,and boundary conditions(BCs)and slenderness ratio of the beam change.Furthermore,a comparative study is conducted between the derived analysis outcomes for BFRC and homogenous polymer beams to examine the effectiveness of bamboo fibers as reinforcement materials,demonstrating the significant enhancements in both vibration and buckling responses,with the exception of natural frequencies for cantilever beams on the Pasternak foundation with the FG-◇fiber distribution.Eventually,the obtained analysis results of BFRC beams are also compared with those for carbon nanotube reinforced composite(CNTRC)beams found in the literature,indicating that the buckling loads and natural frequencies of BFRC beams are lower than those of CNTRC beams.

关键词： bamboo fiber free vibration buckling analysis functionally graded(FG)beam elastic foundation generalized differential quadrature method(GDQM)

Effect of mitomycin C on the corneal endothelial cells of Saudi patients with myopia after transepithelial photorefractive keratectomy:a two-armed cohort study

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International Journal of Ophthalmology(English edition) 2024年第5期17卷 909-915页

作者： Sultan h Alrashidi Department of Ophthalmology College of MedicineQassim UniversityBuraidah 51452Saudi Arabia

AIM:To study the effect of mitomycin C(MMC)applied during transepithelial photorefractive keratectomy(TPRK)on the corneal endothelium one week(W1)and three months(M3)after surgery and its determinants.METhODS:In this two-armed cohort study conducted in 2022,eyes treated with MMC during TPRK(group 1)were compared with eyes not treated with MMC(group 2).The corneal endothelial cell(EC)count,EC density(ECD;cells/mm2),average(μm2),standard deviation(μm2),coefficient of variation(CV%),ECmax,ECmin,and EC percentage of hexagonality were estimated at W1 and M3.The postoperative changes in the EC count in the two groups were compared and correlated with the other independent variables.RESULTS:Group 1 had 26 eyes,and group 2 had 78 eyes.All TPRK indices were significantly higher for the eyes in group 1 than for those in group 2.The MMC usage was not a significant predictor of the change in ECD(P=0.644),change in CV(P=0.374),and change in the percentage of hexagonality of EC(P=0.164)at W1.however,the use of MMC was a significant predictor of changes in CV(P=0.014)and the change in the percentage of hexagonality of EC(P=0.039)at M3.The duration of laser exposure and the size of the optical zone influenced the correlation of MMC use with the changes in EC indices,postoperatively.CONCLUSION:The use of MMC doesn’t affect ECD,CV,and percentage of hexagonality at W1 if other surgical indices are considered.At M3 after operating myopic eyes by TPRK,MMC significantly influence the CV and percentage of hexagonality.The duration of the laser application and the size of the optical zone should be considered when determining the effect of MMC on the EC indices.

关键词： transepithelial photorefractive keratectomy mitomycin-C corneal endothelium

Convexification of hybrid AC-DC Optimal Power Flow with Line-Commutated Converters

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CSEE Journal of Power and Energy Systems 2024年第2期10卷 617-628页

作者： hongyuan Liang Zhigang Li J.h.Zheng Q.h.Wu School of Electric Power Engineering South China University of TechnologyGuangzhou 510641China

Line-commutated converter (LCC)-based high-voltage DC (hVDC) systems have been integrated with bulk AC power grids for interregional transmission of renewable power. The nonlinear LCC model brings additional nonconvexity to optimal power flow (OPF) of hybrid AC-DC power grids. A convexification method for the LCC station model could address such nonconvexity but has rarely been discussed. We devise an equivalent reformulation for classical LCC station models that facilitates second-order cone convex relaxation for the OPF of LCC-based AC-DC power grids. We also propose sufficient conditions for exactness of convex relaxation with its proof. Equivalence of the proposed LCC station models and properties, exactness, and effectiveness of convex relaxation are verified using four numerical simulations. Simulation results demonstrate a globally optimal solution of the original OPF can be efficiently obtained from relaxed model.

关键词： AC-DC power system convex relaxation linecommutated converter optimal power flow second-order cone programming

holographic dark energy in non-conserved gravity theory

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Communications in Theoretical Physics 2024年第4期76卷 126-133页

作者： h R Fazlollahi Institute of Gravitation and Cosmology Peoples Friendship University of Russia(RUDN University)6 Miklukho-Maklaya StMoscow117198Russia

Recently,reconsidering the Rastall ideaT■=a,_(μ) through relativistic thermodynamics gives a new form for the scalar field which led us to construct modern modified theory of gravity debugged'non-conserved gravity theory'Fazlollahi 2023 Euro.Phys.J.C 83923.This theory unlike other modified theories of gravity cannot directly explain the current acceleration expansion in the absence of the cosmological constant and or existence of other forms of dark energy.hence,in this study we have reinvestigated holographic dark energy in the non-conserved theory of gravity.In this context,the density and pressure of dark energy depend on the non-conserved term and density of the dust matter field.As shown,due to non-conservation effects on large-scale structures,unlike the original holographic model,our model onsets an acceleration epoch for the current Universe satisfies observations.Moreover,the interaction and viscous scenarios are studied for this model.

关键词： non-conserved gravity covariant form of thermodynamics holographic dark energy

Marinoan glaciation in the Indian subcontinent——Anatomy and global implications

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Journal of Palaeogeography 2024年第2期13卷 293-308页

作者： h.N.Bhattacharya Department of Earth Sciences Techno India UniversityWest BengalSector VSalt LakeKolkata700091India

Detailed sedimentological analysis of the Pokaran Boulder Bed,representing the most basal unit of the Neoproterozoic-Lower Cambrian Marwar Supergroup,clearly indicates its glacial origin.The glacial sediments are interpreted as an ice-contact submarine fan deposit.Based on the detrital and inherited zircon population of the Marwar Supergroup sediments and interlayered pyroclastic deposits,a Marinoan cryochron of the Cryogenian Period is envisaged for the Pokaran Boulder Bed.The well-preserved Ediacaran elements in the post-glacial sedimentary succession also support a Cryogenian to Early Cambrian age of the Marwar Supergroup.The glacial deposition at the base of the Marwar Supergroup strengthens the regional correlation between the studied sediments with the Lesser himalayan Blaini-Krol-Tal sediments and the haqf Supergroup of Oman.The available zircon ages and paleomagnetic data of the Malani Igneous suite,along with the Marinoan glacial deposits,detrital zircon ages,and Ediacaran fossil elements of the Marwar Supergroup add significant information to the Neoproterozoic Earth history.

关键词： Neoproterozoic Marinoan glaciation Diamictites Pokaran Boulder Bed Marwar Supergroup

Experimental and numerical investigation on alternatives to sandy gravel

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Defence Technology（防务技术） 2024年第1期31卷 130-141页

作者： V.Denefeld h.Aurich Fraunhofer Institute for high-Speed Dynamics Ernst-Mach-Institut(EMI)Ernst-Zermelo-Str.479104FreiburgGermany Fraunhofer Institute for high-Speed Dynamics Ernst-Mach-Institut(EMI)Am Christianswuhr 279400KandernGermany

The NATO agreement STANAG 4569 defines the protection levels for the occupants of logistic and light armored vehicle.The Allied Engineering Publication,AEP-55,Volume 2 document outlines the test conditions for underbelly improvised explosive device(IEDs),which must be buried in water-saturated sandy gravel.The use of sandy gravel has some drawbacks,for instance reproducibility,time consumption,and cost.This paper focuses on the investigation of four alternatives to sandy gravel,which could produce similar specific and cumulative impulses:a concrete pot filled with water,a concrete pot filled with quartz sand,a steel pot without filling and a concrete pot filled with glass spheres(diameter 200μm—300μm)and different water contents.The impulses are measured with a ring technology developed at the Fraunhofer EMI.A numerical soil model based on the work of Marrs,2014 and Fi serov a,2006 and considering the soil moisture was used to simulate the experiments with glass spheres at different water contents,showing much better agreement with the experiments than the classical Laine&Sandvik model,even for high saturation levels.These results can be used to create new test conditions at original scale that are more cost-effective,more reproducible and simpler to manage in comparison to the current tests carried out with STANAG sandy gravel.

关键词： Improvised explosive device(IED) Specific impulse Momentum transfer Sandy gravel Glass spheres Numerical model Soil moisture