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Compound heterozygous mutation of AFG3L2 causes autosomal recessive spinocerebellar ataxia through mitochondrial impairment and MICU1 mediated Ca2+overload

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Science China(life Sciences) 2025年第2期68卷 484-501页

作者： hongyu li Qingwen Ma Yan Xue linlin Cai liwen Bao Lei hong Yitao Zeng Shu-Zhen huang Richard h.Finnell Fanyi Zeng Shanghai Institute of Medical Genetics Shanghai Children’s HospitalShanghai Jiao Tong University School of MedicineShanghai 200040China Department of histo-Embryology Genetics and Developmental BiologyShanghai Jiao Tong University School of MedicineShanghai 200025China nhC Key Laboratory of Medical Embryogenesis and Developmental Molecular Biology Shanghai Key Laboratory of Embryo and Reproduction EngineeringShanghai 200040China Center for Precision Environmental health Baylor College of MedicineHouston 77030USA School of Pharmacy Macao University of Science and TechnologyMacao 999078China

Autosomal recessive spinocerebellar ataxias(SCARs)are one of the most common neurodegenerative diseases characterized by progressive *** SCARs are known to be caused by mutations in multiple genes,there are still many cases that go undiagnosed or are *** this study,we presented a SCAR patient,and identified a probable novel pathogenic mutation(c.1A>G,p.M1V)in the AFG3L2 start *** proband's genotype included heterozygous mutations of the compound AFG3L2(p.[M1V];[R632X](c.[1A>G];[1894.C>T])),which were inherited from the father(c.1A>G,p.M1V)and mother(c.1894C>T,p.R632X).Functional studies performed on hi PSCs(human induced pluripotent stem cells)generated from the patients and hEK293T cells showed that the mutations impair mitochondrial function and the unbalanced expression of AFG3L2 mRnA and protein ***,this novel mutation resulted in the degradation of the protein and the reduction of the stability of the AFG3L2 protein,and MCU(mitochondrial calcium uniporter)complex mediated Ca2+overload.

关键词： AFG3L2 gene autosomal recessive spinocerebellar ataxia mitochondrial impairment Ca2+overload

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Observation of the Y(4230)and a new structure in e^(+)e^(-)→K^(+)K^(-)J/Ψ

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Chinese Physics C 2022年第11期46卷 7-21页

作者： M.Ablikim M.n.Achasov P.Adlarson M.Albrecht R.Aliberti A.Amoroso M.R.An Q.An X.h.Bai Y.Bai O.Bakina R.Baldini Ferroli I.Balossino Y.Ban V.Batozskaya D.Becker K.Begzsuren n.Berger M.Bertani D.Bettoni F.Bianchi J.Bloms A.Bortone I.Boyko R.A.Briere A.Brueggemann h.Cai X.Cai A.Calcaterra G.F.Cao n.Cao S.A.Cetin J.F.Chang W.L.Chang G.Chelkov C.Chen G.Chen h.S.Chen M.L.Chen S.J.Chen T.Chen X.R.Chen X.T.Chen Y.B.Chen Z.J.Chen W.S.Cheng X.Chu G.Cibinetto F.Cossio J.J.Cui h.L.Dai J.P.Dai A.Dbeyssi R.Ede Boer D.Dedovich Z.Y.Deng A.Denig I.Denysenko M.Destefanis F.De Mori Y.Ding J.Dong L.Y.Dong M.Y.Dong X.Dong S.X.Du P.Egorov Y.L.Fan J.Fang S.S.Fang W.X.Fang Y.Fang R.Farinelli L.Fava F.Feldbauer G.Felici C.Q.Feng J.h.Feng K Fischer M.Fritsch C.Fritzsch C.D.Fu h.Gao Y.n.Gao Yang Gao S.Garbolino I.Garzia P.T.Ge C.Geng E.M.Gersabeck A Gilman K.Goetzen L.Gong W.X.Gong W.Gradl M.Greco M.h.Gu C.Y Guan A.Q.Guo L.B.Guo R.P.Guo Y.P.Guo A.Guskov T.T.han W.Y.han X.Q.hao F.A.harris K.K.he K.L.he F.h.heinsius C.h.heinz Y.K.heng C.herold M.himmelreich T.holtmann G.Y.hou Y.R.hou Z.L.hou h.M.hu J.F.hu T.hu Y.hu G.S.huang K.X.huang L.Q.huang L.Q.huang X.T.huang Y.P.huang Z.huang T.hussain n husken W.Imoehl M.Irshad J.Jackson S.Jaeger S.Janchiv Q.Ji Q.P.Ji X.B.Ji X.L.Ji Y.Y.Ji Z.K.Jia h.B.Jiang S.S.Jiang X.S.Jiang Y.Jiang J.B.Jiao Z.Jiao S.Jin Y.Jin M.Q.Jing T.Johansson n.Kalantar-nayestanaki X.S.Kang R.Kappert M.Kavatsyuk B.C.Ke I.K.Keshk A.Khoukaz P.Kiese R.Kiuchi R.Kliemt L.Koch O.B.Kolcu B.Kopf M.Kuemmel M.Kuessner A.Kupsc W.Kuhn J.J.Lane J.S.Lange P.Larin A.Lavania L.Lavezzi Z.h.Lei h.Leithoff M.Lellmann T.Lenz C.li C.li C.h.li Cheng li D.M.li F.li G.li h.li h.li h.B.li h.J.li h.n.li J.Q.li J.S.li J.W.li Ke li L.J li L.K.li Lei li M.h.li P.R.li S.X.li S.Y.li T.li W.D.li W.G.li X.h.li X.L.li Xiaoyu li Z.Y.li h.liang h.liang h.liang Y.F.liang Y.T.liang G.R.liao L.Z.liao J.libby A.limphirat C.X.lin D.X.lin T.lin B.J.liu C.X.liu D.liu F.h.liu Fang liu Feng liu G.M.liu h.liu h.M.liu huanhuan liu huihui liu J.B.liu J.L.liu J.Y.liu K.liu K.Y.L Institute of high Energy Physics Beijing 100049China Beihang University Beijing 100191China Beijing Institute of Petrochemical Technology Beijing 102617China Bochum Ruhr-University D-44780 BochumGermany Carnegie Mellon University PittsburghPennsylvania 15213USA Central China normal University Wuhan 430079China China Center of Advanced Science and Technology Beijing 100190China COMSATS University Islamabad Lahore CampusDefence RoadOff Raiwind Road54000 LahorePakistan Fudan University Shanghai 200433China G.I.Budker Institute of nuclear Physics SB RAS(BInP) Novosibirsk 630090Russia GSI helmholtzcentre for heavy Ion Research Gmbh D-64291 DarmstadtGermany Guangxi normal University Guilin 541004China hangzhou normal University Hangzhou 310036China helmholtz Institute Mainz Staudinger Weg 18D-55099 MainzGermany henan normal University Xinxiang 453007China henan University of Science and Technology Luoyang 471003China henan University of Technology Zhengzhou 450001China huangshan College Huangshan 245000China hunan normal University Changsha 410081China hunan University Changsha 410082China Indian Institute of Technology Madras Chennai 600036India Indiana University BloomingtonIndiana 47405USA InFn Laboratori nazionali di Frascati INFN Laboratori Nazionali di FrascatiI-00044FrascatiItaly InFn Laboratori nazionali di Frascati INFN Sezione di PerugiaI-06100PerugiaItaly InFn Laboratori nazionali di Frascati University of PerugiaI-06100PerugiaItaly InFn Sezione di Ferrara INFN Sezione di FerraraI-44122FerraraItaly InFn Sezione di Ferrara University of FerraraI-44122FerraraItaly Institute of Modern Physics Lanzhou 730000China Institute of Physics and Technology Peace Ave.54BUlaanbaatar 13330Mongolia Jilin University Changchun 130012China Johannes Gutenberg University of Mainz Johann-Joachim-Becher-Weg 45D-55099 MainzGermany Joint Institute for nuclear Research 141980 DubnaMoscow regionRussia Justus-liebig-Universitaet Giessen II.Physikalisches InstitutHeinrich-Buff-Ring 16

The cross sections of e^(+)e^(-)→K^(+)K^(-)J/Ψat center-of-mass energies from 4.127 to 4.600 GeV are measured based on 15.6 fb-1data collected with the BESⅢ detector operating at the BEPCⅡ storage *** resonant structures are observed in the line shape of the cross *** mass and width of the first structure are measured to be(4225.3±2.3±21.5)MeV and(72.9±6.1±30.8)MeV,*** are consistent with those of the established Y(4230).The second structure is observed for the first time with a statistical significance greater than 8σ,denoted as Y(4500).Its mass and width are determined to be(4484.7±13.3±24.1)MeV and(111.1±30.1±15.2)MeV,*** first presented uncertainties are statistical and the second ones are *** product of the electronic partial width with the decay branching fractionΓ(Y(4230)→e^(+)e^(−))B(Y(4230)→K^(+)K^(−)J/Ψ)is reported.

关键词： Y states charmonium-like states BESⅢ

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The Tibetan Plateau darkening initiates positive feedback through the Arctic

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Science Bulletin 2025年第3期70卷 299-300页

作者： Laurent li Laboratoire de Météorologie Dynamique Centre National de la Recherche ScientifiqueSorbonne UniversitéParis 75005France

With its high elevation and its geographic location in the subtropics,the Tibetan Plateau exerts strong mechanic forcing and thermodynamic control on the atmospheric general circulation,and consequently on climate var... 详细信息

关键词： Tibetan Plateau Arctic

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Luminosities and energies of e^(+)e^(−) collision data taken between √s=4.61 GeV and 4.95 GeV at BESⅢ

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Chinese Physics C 2022年第11期46卷 64-75页

From December 2019 to June 2021,the BESⅢ experiment collected approximately 5.85 fb^(−1) of data at center-of-mass energies between 4.61 and 4.95 *** is the highest collision energy BEPCⅡ has reached to *** accumulated e^(+)e^(−) annihilation data samples are useful for studying charmonium(-like)states and charmed-hadron *** adopting a novel method of analyzing the production of A_(c)^(+)A_(c)^(-) pairs in e^(+)e^(−) annihilation,the center-of-mass energies are measured with a precision of 0.6 *** luminosities are measured with a precision of better than 1% by analyzing the events of large-angle Bhabha *** measurements provide important inputs to analyses based on these data samples.

关键词： luminosity center-of-mass energy BESⅢdetector

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Characteristics and phytotoxicity of hydrochar-derived dissolved organic matter:Effects of feedstock type and hydrothermal temperature

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Journal of Environmental Sciences 2025年第3期149卷 139-148页

作者： Qianqian Lang Xuan Guo Chao Wang lingyao li Yufei li Junxiang Xu Xiang Zhao Jijin li Bensheng liu Qinping Sun Guoyuan Zou Institute of Plant nutrition and Resources Beijing Academy of Agriculture and Forestry SciencesBeijing 100097China

The dissolved organic matter(DOM)with high mobility and reactivity plays a crucial role in *** this study,the characteristics and phytotoxicity ofDOMreleased fromthe hydrochars prepared from different feedstocks(cowmanure,corn stalk and Myriophyllum aquaticum)under three hydrothermal carbonization(hTC)temperatures(180,200 and 220°C)were *** results showed that the hydrochars had high dissolved organic carbon content(20.15 to 37.65 mg/g)and its content showed a gradual reduction as hTC temperature *** fluorescent components including mixed substance of fulvic acid-like and humic acid-like substances(C1,30.92%-58.32%),UVA humic acid-like substance(C2,25.27%-29.94%)and protein-like substance(C3,11.74%-41.92%)were identified in hydrochar DOM by excitation emission matrix spectra coupled with parallel factor *** hTC temperature increased the relative proportion of aromatic substances(C1+C2)and humification degree of hydrochar DOM from cow manure,while it presented adverse effects on the hydrochar DOM from corn stalk and *** principal component analysis suggested that feedstock type and hTC temperature posed significant effects on the characteristics of hydrochar ***,seed germination test of all hydrochar DOM demonstrated that the root length was reduced by 8.88%-26.43%in contrast with control,and the germination index values were 73.57%-91.12%.These findings provided new insights into the potential environmental effects for hydrochar application in soil.

关键词： hydrochar hydrothermal temperature Dissolved organic matter Excitation emission matrix Parallel factor analysis Phytotoxicity

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Multilevel analysis of the central-peripheral-target organ pathway:contributing to recovery after peripheral nerve injury

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neural Regeneration Research 2025年第10期20卷 2807-2822页

作者： Xizi Song Ruixin li Xiaolei Chu Qi li Ruihua li Qingwen li Kai-Yu Tong Xiaosong Gu Dong Ming Academy of Medical Engineering and Translational Medicine Tianjin UniversityTianjinChina haihe Laboratory of Brain-Machine Interface and human-Machine Fusion TianjinChina Department of Rehabilitation Tianjin University Tianjin HospitalTianjinChina Department of hand Microsurgery Tianjin University Tianjin HospitalTianjinChina School of Exercise and health Tianjin University of SportTianjinChina Department of Biomedical Engineering the Chinese University of Hong KongHong Kong Special Administrative RegionChina

Peripheral nerve injury is a common neurological condition that often leads to severe functional limitations and *** on the pathogenesis of peripheral nerve injury has focused on pathological changes at individual injury sites,neglecting multilevel pathological analysis of the overall nervous system and target *** has led to restrictions on current therapeutic *** this paper,we first summarize the potential mechanisms of peripheral nerve injury from a holistic perspective,covering the central nervous system,peripheral nervous system,and target *** peripheral nerve injury,the cortical plasticity of the brain is altered due to damage to and regeneration of peripheral nerves;changes such as neuronal apoptosis and axonal demyelination occur in the spinal *** nerve will undergo axonal regeneration,activation of Schwann cells,inflammatory response,and vascular system regeneration at the injury *** damage to target organs can occur,including skeletal muscle atrophy and sensory receptor *** then provide a brief review of the research advances in therapeutic approaches to peripheral nerve *** main current treatments are conducted passively and include physical factor rehabilitation,pharmacological treatments,cell-based therapies,and physical ***,most treatments only partially address the problem and cannot complete the systematic recovery of the entire central nervous system-peripheral nervous system-target organ ***,we should further explore multilevel treatment options that produce effective,long-lasting results,perhaps requiring a combination of passive(traditional)and active(novel)treatment methods to stimulate rehabilitation at the central-peripheral-target organ levels to achieve better functional recovery.

关键词： central nervous system central peripheral target organ multilevel pathological analysis nerve regeneration peripheral nerve injury peripheral nervous system target organs therapeutic approach

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Alpha-decay properties of nuclei around neutron magic numbers

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nuclear Science and Techniques 2025年第1期36卷 140-149页

作者： Ming li Chu-Xin Chen Lan-Fang Xiao Yi Zhang Song Luo Xiao-hua li School of nuclear Science and Technology University of South ChinaHengyang 421001China PET‑CT Center Chenzhou First People’s HospitalChenzhou 423000China hengyang Ecological Environment Monitor Center Hengyang 421001China China Certification and Inspection Group Hefei 230000China national Exemplary Base for International Science and Technology Collaboration of nuclear Energy and nuclear Safety University of South ChinaHengyang 421001China Cooperative Innovation Center for nuclear Fuel Cycle Technology and Equipment University of South ChinaHengyang 421001China Key Laboratory of Low Dimensional Quantum Structures and Quantum Control Hunan Normal UniversityChangsha 410081China

By combining experimental α-decay energies and half-lives, the α-particle preformation factor for nuclei around neutron magic numbers n of 126, 152, and 162 were extracted using the two-potential approach. The nuclei around the shell closure were more tightly bound than adjacent nuclei. Additionally, based on the WS4 mass model (Wang et al., Phys. Lett.B 734, 215 (2014)), we extended the two-potential approach to predict the α-decay half-lives of nuclei around n values of178 and 184 with Z of 119 and 120. We believe that our findings will serve as guidelines for future experimental studies.

关键词： adecay Preformation factor neutron magic number heavy and superheavy nuclei

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Targets capture by distributed active swarms via bio-inspired reinforcement learning

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Science China(Physics,Mechanics & Astronomy) 2025年第1期68卷 206-217页

作者： Kun Xu Yue li Jun Sun Shuyuan Du Xinpeng Di Yuguang Yang Bo li Institute of Biomechanics and Medical Engineering Applied Mechanics LaboratoryDepartment of Engineering MechanicsTsinghua UniversityBeijing 100084China Institute of nuclear and new Energy Technology Tsinghua UniversityBeijing 100084China Shanghai Aerospace Control Technology Institute Shanghai 201109China Shanghai Key Laboratory of Aerospace Intelligent Control Technology Shanghai 201109China College of Automation Engineering Nanjing University of Aeronautics and AstronauticsNanjing 210016China

natural swarms arranged from cells to herds are usually decentralized but display intriguing collective intelligence in coordinating individuals across large scales to efficiently achieve their common *** from nature may provide new strategies for controlling collective dynamics of synthetic swarms to accomplish specific ***,we present a bioinspired computational framework that steers distributed active swarms to collectively capture and merge targets via reinforcement *** exploit collective milling structures of natural herds to cage the targets,and adopt a switching control policy inspired by sperms’chiral dynamics to optimize the trajectories of individuals,through which the active swarms can selforganize to enclose single or multiple distant targets in a dynamical,adaptive and scalable *** exists a critical swarm size,beyond which the excessive competition between agents would generate large mechanical forces,leading to capture instability but enabling the transition from short-distance to long-distance merging capture of multiple *** work provides physical insights into distributed active swarms and could offer a multilevel,decentralized strategy toward controlling swarm robotics in wide applications such as bio-medical devices,machine immunity,and target clearance.

关键词： swarm intelligence robotics multi-tasks artificial intelligence bionics

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Darboux transformation,positon solution,and breather solution of the third-order flow Gerdjikov–Ivanov equation

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Chinese Physics B 2025年第1期34卷 195-202页

作者： Shuzhi liu ning-Yi li Xiaona Dong Maohua li School of Statistics and Data Science Ningbo University of TechnologyNingbo 315211China School of Mathematics and Statistics Ningbo UniversityNingbo 315211China College of Wealth and Management Ningbo University of Finance and EconomicsNingbo 315175China

The third-order flow Gerdjikov–Ivanov(TOFGI)equation is studied,and the Darboux transformation(DT)is used to obtain the determinant expression of the solution of this *** this basis,the soliton solution,rational solution,positon solution,and breather solution of the TOFGI equation are obtained by taking zero seed solution and non-zero seed *** exact solutions and dynamic properties of the Gerdjikov–Ivanov(GI)equation and the TOFGI equation are compared in detail under the same conditions,and it is found that there are some differences in the velocities and trajectories of the solutions of the two equations.

关键词： third-order flow Gerdjikov-Ivanov equation solitons positons breathers

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Quantum public-key crypto via EPR pairs

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Science China(Physics,Mechanics & Astronomy) 2025年第1期68卷 54-59页

作者： Xiang-Qun Fu hong-Wei li Jian-hong Shi Tan li Wan-Su Bao henan Key Laboratory of Quantum Information and Cryptography Information Engineering UniversityZhengzhou 450004China Synergetic Innovation Center of Quantum Information and Quantum Physics University of Science and Technology of ChinaHefei 230026China

With the development of quantum computing technology,quantum public-key cryptography is gradually becoming an alternative to the existing classical public-key *** paper designs a quantum trapdoor one-way function via EPR pairs and quantum *** on this,a new quantum public-key cryptosystem is presented,which offers forward security,and can resist the chosen-plaintext attack and chosen-ciphertext *** with the existing quantum public-key cryptos,eavesdropping can be automatically detected in this new quantum public-key cryptosystem under a necessary condition,which is also detailed in the paper.

关键词： quantum public-key crypto trapdoor one-way function EPR pairs

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