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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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B2-CuZr reinforced amorphous alloy matrix composites:A review

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China Foundry 2025年第1期22卷 1-11页

作者： Wei Guo Run-hua huang zhen Zhang Mi Zhao Jin-cheng Wang Yan-qiang Qiao shu-lin Lü shu-sen Wu State Key Lab of Materials Processing and Die&Mould Technology School of Materials Science and EngineeringHuazhong University of Science and TechnologyWuhan 430074China State Key Laboratory of Solidification Processing Northwestern Polytechnical UniversityXi’an 710072China School of Aerospace Engineering Huazhong University of Science and TechnologyWuhan 430074China Research Institute of Huazhong University of Science and Technology in Shenzhen Shenzhen 518057GuangdongChina

B2-CuZr phase reinforced amorphous alloy matrix composites has become one of the research hotspots in the field of materials science due to the“transformation-induced plasticity”phenomenon,which makes the composites show better macroscopic plastic deformability and obvious work-hardening behavior compared to the conventional amorphous alloy matrix composites reinforced with ductile ***,the in-situ metastable B2-CuZr phase tends to undergo eutectoid decomposition during solidification,and the volume fraction,size,and distribution of B2-CuZr phase are difficult to control,which limits the development and application of these *** date,much efforts have been made to solve the above problems through composition optimization,casting parameter tailoring,and post-processing *** this study,a review was given based on relevant studies,focusing on the predictive approach,reinforcing mechanism,and microstructure tailoring methods of B2-CuZr phase reinforced amorphous alloy matrix *** research focus and future prospects were also given for the future development of the present composite system.

关键词： amorphous alloy matrix composite B2-CuZr phase plasticity microstructure tailoring

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Effect of two-pass rolling of textured roll and polished roll on surface topography and mechanical properties of 316L stainless steel ultra-thin strip

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Journal of Iron and Steel Research International 2025年第1期32卷 186-197页

作者： Zeng-qiang Zhang Xi Liao Zhong-kai Ren zhen-hua Wang Ya-xing Liu Tao Wang Qing-xue huang College of Mechanical and Vehicle Engineering Taiyuan University of TechnologyTaiyuan030024ShanxiChina Engineering Research Center of Advanced Metal Composites Forming Technology and Equipment of Ministry of Education Taiyuan University of TechnologyTaiyuan030024ShanxiChina National Key Laboratory of Metal Forming Technology and Heavy Equipment Taiyuan University of TechnologyTaiyuan030024ShanxiChina Shanxi TISCO Stainless Steel Precision Strip Co. Ltd.Taiyuan030032ShanxiChina

The textured roll and polished roll were applied instead of the ground roll in a 20-high mill to conduct two-pass rolling of 316L stainless steel strip with thickness of 0.027 *** the two-pass rolling with the textured roll and polished roll(TPR),the surface roughness of the strip is dramatically reduced,and the surface topographical anisotropy index is diminished to 30.9%of the initial *** with the strip rolled using the ground roll in both passes(GGR),the elongation of TPR rolled strip is obviously improved,and the mechanical property anisotropy is greatly *** anisotropy index of tensile strength and elongation are 42.58%and 52.59%of that of GGR rolled strip,which is mainly attributed to the significant decrease of the texture intensity of the strip by TPR *** results indicate that TPR process can obtain the stainless steel ultra-thin strip with smooth and uniform surface topography and good mechanical properties.

关键词： Textured roll Polished roll Surface topography Mechanical property Stainless steel ultra-thin strip

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Is the Gum Nebula an important interstellar scattering disk of background pulsars?

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Science China(Physics,Mechanics & Astronomy) 2025年第3期68卷 175-186页

作者： Rui Wang zhen Yan Zhiqiang Shen Ke Jia Lee Yajun Wu Rongbing Zhao Zhipeng huang Xiaowei Wang Jie Liu Shanghai Astronomical Observatory Chinese Academy of SciencesShanghai200030China School of Astronomy and Space Sciences University of Chinese Academy of SciencesBeijing100049China Key Laboratory of Radio Astronomy and Technology Chinese Academy of SciencesBeijing100101China School of Physical Science and Technology ShanghaiTech UniversityShanghai201210China Department of Astronomy School of PhysicsPeking UniversityBeijing100871China Kavli Institute for Astronomy and Astrophysics Peking UniversityBeijing100871China Department of Physics and Astronomy Hubei University of EducationWuhan430205China

The Gum Nebula is a faint supernova remnant extending about 40°across the southern sky,potentially affecting tens of background *** the view that the Gum Nebula acts as a potential scattering screen for background pulsars has been recurrently mentioned over the past five decades,it has not been directly *** chose the strong background pulsar PSR B0740-28 as a probe and monitored its diffractive interstellar scintillation(DISS)at 2.25&8.60 GHz simultaneously for about two years using the Shanghai Tian Ma Radio Telescope(TMRT).DISS was detected at both frequencies and quantified by two-dimensional autocorrelation *** calculated their scattering spectral indexαand found that 9/21 of the observations followed the theoretical predictions,while 4/21 of them clearly showedα<*** finding provides strong support for anomalous scattering along the pulsar line of sight,due to the large frequency lever arm and the simultaneous features of our dual-frequency *** comparison to the 2.25 GHz observations,scintillation arcs were observed in 10/21 of the secondary spectrum plots for 8.60 GHz ***,the highest frequency record for pulsar scintillation arc detection was updated to 8.60 *** fitting results were the most direct evidence for the view that the Gum Nebula acts as the scattering screen for background pulsars,because both the distance(245_(-72)^(+69)pc)and transverse speed(22.4_(-4.2)^(+4.1)km s^(-1))of the scintillation screen are comparable with related parameters of the Gum *** findings indicated that anisotropic scattering provides a superior explanation for the annual modulation of scintillation arcs than isotropic ***,the orientation of its long axis was also fitted.

关键词： pulsar interstellar scintillation Gum Nebula

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Spatially confined synthesis of TiNb_(2)O_(7)quantum dots onto mesoporous carbon and Ti_(3)C_(2)T_(X)MXene for boosting lithium storage

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Green Energy & Environment 2025年第2期10卷 358-366页

作者： Daoguang Sun Cheng Tang Haitao Li Xinlin Zhang Guanjia Zhu zhen-Dong huang Aijun Du Haijiao Zhang Institute of Nanochemistry and Nanobiology School of Environmental and Chemical EngineeringShanghai UniversityShanghai200444China School of Chemistry and Physics and Centre for Materials Science Queensland University of TechnologyBrisbaneQLD4001Australia State Key Laboratory for Organic Electronics and Information Displays&Jiangsu Key Laboratory for Biosensors Institute of Advanced Materials(IAM)Jiangsu National Synergetic Innovation Center for Advanced Materials(SICAM)Nanjing University of Posts and Telecommunications9 Wenyuan RoadNanjing210023China

TiNb_(2)O_(7)has been emerged as one of the most promising electrode materials for high-energy lithium-ion ***,limited by the slow electron/ion transport kinetics,and insufficient active sites in the bulk structure,the TiNb_(2)O_(7)electrode still suffers from unsatisfactory lithium storage ***,we demonstrate a spatially confined strategy toward a novel TiNb_(2)O_(7)-NMC/MXene composite through a triblock copolymer-directed one-pot solvothermal route,where TiNb_(2)O_(7)quantum dots with a particle size of 2-3 nm are evenly embedded into N-doped mesoporous carbon(NMC)and Ti_(3)C_(2)T_(X)***,the as-prepared TiNb_(2)O_(7)-NMC/MXene anode exhibits a high reversible capacity(486.2 mAh g^(-1)at 0.1 A g^(-1)after 100 cycles)and long cycle lifespan(363.4 mAh g^(-1)at ss1 A g^(-1)after 500 cycles).Both experimental and theorical results further demonstrate that such a superior lithium storage performance is mainly ascribed to the synergistic effect among 0D TiNb_(2)O_(7)quantum dots,2D Ti_(3)C_(2)T_(X)MXene nanosheets,and N-doped mesoporous *** strategy presented also opens up new horizon for space-confined preparation of high-performance electrode materials.

关键词： TiNb_(2)O_(7)quantum dots Ti_(3)C_(2)T_(X)MXene Mesoporous carbon Confined synthesis Lithium-ion batteries

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Metallized polymer current collector as “stress acceptor” for stable micron-sized silicon anodes

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Journal of Energy Chemistry 2025年第2期101卷 786-794页

作者： Ziyi Cao Haoteng Sun Yi Zhang Lixia Yuan Yaqi Liao Haijin Ji shuaipeng Hao zhen Li Long Qie Yunhui huang State Key Laboratory of Material Processing and Die & Mold Technology School of Materials Science and Engineering Huazhong University of Science and Technology Department of Chemistry Brown University

Micron-sized silicon（μSi） is a promising anode material for next-generation lithium-ion batteries due to its high specific capacity,low cost,and abundant ***,the volume expansion that occurs during cycling leads to the accumulation of undesirable stresses,resulting in pulverization of silicon microparticles and shortened lifespan of the ***,a composite film of Cu-PET-Cu is proposed as the current collector（CC） for μSi anodes to replace the conventional Cu ***-PET-Cu CC is prepared by depositing Cu on both sides of a polyethylene terephthalate（PET） *** PET layer promises good ductility of the film,permitting the Cu-PET-Cu CC to accommodate the volumetric changes of silicon microparticles and facilitates the stress release through ductile *** a result,the μSi electrode with Cu-PET-Cu CC retains a high specific capacity of 2181 mA h g-1,whereas the μSi electrode with Cu CC（μSi/Cu） exhibits a specific capacity of 1285 mA h g-1after 80 *** stress relieving effect of CuPET-Cu was demonstrated by in-situ fiber optic stress monitoring and multi-physics *** work proposes an effective stress relief strategy at the electrode level for the practical implementation of μSi anodes.

关键词： Electrode design Metallized polymer current collector Micron-sized Si anodes Stress relieving

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Pharmacological Mechanism of Chinese Medicine in Systemic Lupus Erythematosus:A Narrative Review

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Chinese Journal of Integrative Medicine 2025年第2期31卷 157-169页

作者： ZHU Bo-yu LIU Zhi-chao ZHAO zhen-xi huang Hui-ping ZHANG Na XIA Jia CHEN Wei-wei Department of Rheumatology Shanghai Municipal Hospital of Traditional Chinese MedicineShanghai University of Traditional Chinese MedicineShanghai(200071)China

Systemic lupus erythematosus(SLE)is a chronic autoimmune disorder affecting multiple systems,characterized by the development of harmful autoantibodies and immune complexes that lead to damage in organs and *** medicine(CM)plays a role in mitigating complications,enhancing treatment effectiveness,and reducing toxicity of concurrent medications,and ensuring a safe ***,CM mainly solves the disease comprehensively through multi-target and multi-channel regulation process,therefore,its treatment mechanism is often complicated,involving many molecular *** review introduces the research progress of pathogenesis of SLE from the aspects of genetics,epigenetics,innate immunity and acquired immunity,and then discusses the molecular mechanism and target of single Chinese herbal medicine and prescription that are commonly used and effective in clinic to treat SLE.

关键词： systemic lupus erythematosus Chinese medicine natural extracts treatment review

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Densification of ceria-based barrier layer for solid oxide cells at lower sintering temperatures:A review

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Journal of Advanced Ceramics 2025年第1期14卷 23-48页

作者： Shiyi Wu Wenyi Yan Na Ni Lei Zhu zhen huang Key Laboratory for Power Machinery and Engineering of Ministry of Education Shanghai Jiao Tong UniversityShanghai 200240China School of Physics and Astronomy Shanghai Jiao Tong UniversityShanghai 200240China

Solid oxide cells(SOCs),including solid oxide fuel cells(SOFCs)and solid oxide electrolysis cells(SOECs),hold great potential for commercial viability because of their high heat utilization,reduced carbon emissions,high efficiency,and fuel flexibility.A key component in enhancing cell performance and extending lifetime is the thin and dense ceria-based barrier layer at the electrolyte and air electrode interface to avoid cell degradation during *** barrier layer needs to be densified at low temperatures(ideally less than 1000℃),which remains a significant *** review focuses on a typical low-cost ceramic powder-based route for the preparation of a barrier layer and explores various approaches to achieve low-temperature *** covers techniques such as synthesizing finer powders,optimizing deposition methods for the green-body layer,using sintering aids,and employing post-sintering processes to increase *** advancements in these areas are highlighted to provide insights into future development ***,the review discusses new opportunities and emerging techniques that could further improve the barrier layer performance.

关键词： solidoxidecells(SOCs) ceria-basedmaterial barrierlayer low-temperaturesintering densification

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A promising approach for quantifying focal stroke modeling and assessing stroke progression:optical resolution photoacoustic microscopy photothrombosis

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Neural Regeneration Research 2025年第7期20卷 2029-2037页

作者： Xiao Liang Xingping Quan Xiaorui Geng Yujing huang Yonghua Zhao Lei Xi zhen Yuan Ping Wang Bin Liu Faculty of Health Sciences University of MacaoMacao Special Administrative RegionChina Center for Cognitive and Brain Sciences University of MacaoMacao Special Administrative RegionChina Department of Biomedical Engineering Southern University of Science and TechnologyShenzhenGuangdong ProvinceChina State Key Laboratory of Quality Research in Chinese Medicine Institute of Chinese Medical SciencesUniversity of MacaoTaipaMacao Special Administrative RegionChina Department of Emergency Zhujiang HospitalSouthern Medical UniversityGuangzhouGuangdong ProvinceChina

To investigate the mechanisms underlying the onset and progression of ischemic stroke,some methods have been proposed that can simultaneously monitor and create embolisms in the animal cerebral ***,these methods often require complex systems and the effect of age on cerebral embolism has not been adequately studied,although ischemic stroke is strongly *** this study,we propose an optical-resolution photoacoustic microscopy-based visualized photothrombosis methodology to create and monitor ischemic stroke in mice simultaneously using a 532 nm pulsed *** observed the molding process in mice of different ages and presented age-dependent vascular embolism ***,we integrated optical coherence tomography angiography to investigate age-associated trends in cerebrovascular variability following a *** imaging data and quantitative analyses underscore the differential cerebrovascular responses to stroke in mice of different ages,thereby highlighting the technique's potential for evaluating cerebrovascular health and unraveling age-related mechanisms involved in ischemic strokes.

关键词： age-dependent cerebral cortex ischemic stroke mouse model optical coherence tomography angiography photoacoustic microscopy photothrombosis vascular imaging

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Significance of monitoring imatinib plasma concentration in secondline treatment decisions for c-kit 11 gene-mutated gastrointestinal stromal tumors

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World Journal of Gastrointestinal Oncology 2025年第3期17卷 81-90页

作者： Hai-Tao Li Yun-Yun Du zhen huang Jin-Jin Li Jun Zhang Department of Gastrointestinal Surgery Nanchuan Hospital of Chongqing Medical UniversityChongqing 408400China Department of Oncology Nanchuan Hospital of Chongqing Medical UniversityChongqing 408400China Department of Gastrointestinal Surgery The First Affiliated Hospital of Chongqing Medical UniversityChongqing 400016China

BACKGROUND For patients with advanced gastrointestinal stromal tumors(GISTs)carrying the ckit exon 11 mutation,imatinib(IM)at a standard dosage of 400 mg per day is the preferred first-line *** cases where treatment with IM fails,there is an urgent need for a more precise assessment method to determine whether to switch therapies or escalate the IM *** approach will enhance clinical decision-making and optimize patient *** To investigate IM plasma concentration’s role in second-line treatment decisions for c-kit 11-mutated advanced GISTs post-IM *** Patients with advanced GIST harboring c-kit 11 mutation who experienced failure with IM 400 mg per day as first-line treatment at our hospital were retrospectively *** were categorized into a low plasma(LP)concentration group(LP group,0.05).For the HP group(n=47),Group B(n=18)had a higher DCR and longer PFS than Group A(n=29)(P=0.008 and P=0.03,respectively).No difference in OS was observed between the two subgroups(P>0.05).CONCLUSION Increasing IM dosage for c-kit 11-mutated advanced GISTs post-IM failure may prolong OS if plasma concentration is<1100 ng/*** tyrosine kinase inhibitors may improve DCR and PFS if≥1100 ng/mL.

关键词： Gastrointestinal stromal tumor C-kit exon 11 Imatinib Plasma concentration Second-line treatment

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