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Lama1 upregulation prolongs the lifespan of the dyH/dyH mouse model of LAMA2-related congenital muscular dystrophy

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j.urnal of Genetics and Genomics 2024年第10期51卷 1066-1078页

作者： Liu Y. Tan D. Ma K. Luo H. Mao j. Luo j. Shen Q. x. L. Yang S. Ge L. Guo Y. zhang H. x.ong H. Department of Pediatrics Peking University First Hospital Beijing 102600 China State Key Laboratory of Vascular Homeostasis and Remodeling The Institute of Cardiovascular Sciences School of Basic Medical Sciences Peking University Health Science Center Beijing 100191 China Department of Neurology The First Affiliated Hospital Jiangxi Medical College Nanchang University Jiangxi Nanchang 330006 China Bio-x.Institutes Key Laboratory for the Genetics of Developmental and Neuropsychiatric Disorders Ministry of Education Shanghai Jiao Tong University Shanghai 200030 China Department of Genetics Yale School of Medicine New Haven 06510 CT United States Medical and Health Analysis Center Peking University Beijing 100191 China Department of Neurology Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China

LAMA2-related congenital muscular dystrophy (LAMA2-CMD), characterized by laminin-α2 deficiency, is debilitating and ultimately fatal. To date, no effective therapy has been clinically available. Laminin-α1, which shares significant similarities with laminin-α2, has been proven as a viable compensatory modifier. To evaluate its clinical applicability, we establish a Lama2 ex.n-3-deletion mouse model (dyH/dyH). The dyH/dyH mice ex.ibit early lethality and typical LAMA2-CMD phenotypes, allowing the evaluation of various endpoints. In dyH/dyH mice treated with synergistic activation mediator-based CRISPRa-mediated Lama1 upregulation, a nearly doubled median survival is observed, as well as improvements in weight and grip. Significant therapeutical effects are revealed by MRI, serum biochemical indices, and muscle pathology studies. Treating LAMA2-CMD with LAMA1 upregulation is feasible, and early intervention can alleviate symptoms and ex.end lifespan. Additionally, we reveal the limitations of LAMA1 upregulation, including high-dose mortality and non-sustained ex.ression, which require further optimization in future studies. © 2024 Institute of Genetics and Developmental Biology, Chinese Academy of Sciences, and Genetics Society of China

关键词： Congenital muscular dystrophy CRISPRa Disease model Gene therapy LAMA2

Multi-instance partial-label learning: towards ex.loiting dual inex.ct supervision

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Science China(Information Sciences) 2024年第3期67卷 48-61页

作者： Wei TANG Weij.a zhang Min-Ling zhang School of Computer Science and Engineering Southeast University Key Laboratory of Computer Network and Information Integration (Southeast University) Ministry of Education School of Information and Physical Sciences The University of Newcastle

Weakly supervised machine learning algorithms are able to learn from ambiguous samples or labels, e.g., multi-instance learning or partial-label learning. However, in some real-world tasks, each training sample is associated with not only multiple instances but also a candidate label set that contains one groundtruth label and some false positive labels. Specifically, at least one instance pertains to the ground-truth label while no instance belongs to the false positive labels. In this paper, we formalize such problems as multi-instance partial-label learning(MIPL). Ex.sting multi-instance learning algorithms and partial-label learning algorithms are suboptimal for solving MIPL problems since the former fails to disambiguate a candidate label set, and the latter cannot handle a multi-instance bag. To address these issues, a tailored algorithm named MIPLGP, i.e., multi-instance partial-label learning with Gaussian processes, is *** first assigns each instance with a candidate label set in an augmented label space, then transforms the candidate label set into a logarithmic space to yield the disambiguated and continuous labels via an ex.lusive disambiguation strategy, and last induces a model based on the Gaussian processes. Ex.erimental results on various datasets validate that MIPLGP is superior to well-established multi-instance learning and partial-label learning algorithms for solving MIPL problems.

关键词： machine learning multi-instance partial-label learning multi-instance learning partial-label learning Gaussian processes

Porous Indium Nanocrystals on Conductive Carbon Nanotube Networks for High-Performance CO2-to-Formate Electrocatalytic Conversion

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Energy & Environmental Materials 2024年第4期7卷 413-421页

作者： Liangping x.ao Rusen Zhou Tianqi zhang x.aox.ang Wang Renwu Zhou Patrick j.Cullen Kostya (Ken) Ostrikov Department of Physics Research Institute for Biomimetics and Soft Matter Fujian Provincial Key Laboratory for Soft Functional Materials Jiujiang Research Institute Xiamen University School of Chemistry and Physics QUT Centre for Materials Science and QUT Centre for a Waste-Free World Queensland University of Technology(QUT) School of Chemical and Biomolecular Engineering University of Sydney

Ever-increasing emissions of anthropogenic carbon diox.de （CO2） cause global environmental and climate *** by biological photosynthesis,developing effective strategies NeuNlto up-cycle CO2into high-value organics is *** CO2reduction reaction （CO2RR） is highly promising to convert CO2into economically viable carbon-based chemicals or fuels under mild process conditions.

关键词： CO2RR conductive network electrocatalysts formate

Effect of samarium doping on the activity and sulfur resistance of Ce/MnFeO_(x. catalyst for low-temperature selective catalytic reduction of NO_(x. by ammonia

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Chinese j.urnal of Chemical Engineering 2024年第7期71卷 272-282页

作者： Qiyao zhang Shuangshuang zhang x. Hu Yongmin Huang Key Laboratory of Specially Functional Polymeric Materials and Related Technology School of Chemistry and Molecular EngineeringEast China University of Science and TechnologyShanghai 200237China

The self-made MnFeO_(x. catalysts doped with cerium and samarium were prepared by impregnation method for low-temperature selective catalytic reduction(SCR)by *** this work,the surface properties of the series of MnFe-based catalysts were *** results indicate Sm-modified catalyst have superior low-temperature SCR activity;NO_(x. conversion maintained at nearby to 100%at 90℃ to 240℃.In addition,The N_(2) selectivity of Sm doping remains above 80%in the range of 60℃ to 150℃.In SO_(2) poisoning test,the NO_(x. conversion can be remained>90%after 10 h of *** x.S,NH_(3)-TPD and H_(2)-TPR results show the catalyst with Sm doping enhances the acid sites and ox.dation catalytic sites of mix.d ox.des serves for improving ox.gen vacancies and transfer *** situ diffuse reflax.ons infrared Fourier transformations spectroscopy(DRIFTS)results show that NO_(x. is more easily adsorbed on the surface after Sm doping,which provided favorable conditions for the NH_(3)-SCR reaction to *** reaction at the catalyst surface will follow the L-H reaction mechanism by transient reaction test.

关键词： SCR Sm doping Catalyst Sulfur resistance In situ DRIFTS Selectivity

Comparative Study on Different Methods for Prediction of Thermal Insulation Performance of Thermal Barrier Coating Used on Turbine Blades

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j.urnal of Thermal Science 2024年第1期33卷 172-189页

作者： zhang Zhix.n ZENG Wu zhang x.aodong ZENG Yuntao Advanced Gas Turbine Laboratory Institute of Engineering ThermophysicsChinese Academy of SciencesBeijing 100190China Key Laboratory of Advanced Energy and Power Institute of Engineering ThermophysicsChinese Academy of SciencesBeijing 100190China Innovation Academy for Light-duty Gas Turbine Chinese Academy of SciencesBeijing 100190China University of Chinese Academy of Sciences Beijing 100049China

As turbine inlet temperature gets higher and higher,thermal barrier coating(TBC) is more and more widely used in turbine *** turbine blades with TBC,it is of great significance to evaluate the temperature distribution of its substrate metal quickly and accurately,especially during the design *** different degrees of simplification such as whether to consider the change of the geometric size of the fluid domain by TBC and whether to consider the planar heat conduction in TBC,three different methods used in conj.gate heat transfer(CHT) simulation to model the TBC of the turbine blades have been developed and widely used by ***,little research has been conducted to investigate the influence of the three methods on the temperature distribution of turbine *** fill this gap,three geometric models were *** are a solid conduction model with a substrate metal layer and a TBC layer,a transonic turbine vane with internal cooling and TBC,and a plate cylindrical film hole cooling model with *** methods were used in these geometric models and their differences were carefully analyzed and *** result shows that for the conduction model used in this paper,with the same TBC surface temperature distribution,the difference between the three methods is very small and can be *** a transonic turbine vane with internal cooling,regarding the local max.mum temperature of the substrate-TBC interface,the largest difference between the method in which TBC is considered as a thermal resistance or a virtual layer of cells and the method in which three-dimensional heat conduction equation of TBC is solved occurs at the trailing *** difference near the leading edge is below *** employed to the film cooling model,the difference of the laterally averaged temperature of the substrate-TBC interface can be 8 K which is mainly due to the change of the length to diameter ratio of the film cooling hole by *** the substrate

关键词： turbine blade thermal barrier coating conj.gate heat transfer film cooling hole

Microstructure evolution and strengthening mechanism of high -performance powder metallurgy TA15 titanium alloy by hot rolling

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International j.urnal of Minerals,Metallurgy and Materials 2024年第6期31卷 1426-1436页

作者： Ying Gao Ce zhang j.azhen zhang x.n Lu Beij.ng Advanced Innovation Center for Materials Genome Engineering State Key Laboratory for Advanced Metals and MaterialsInstitute for Advanced Ma-terials and TechnologyUniversity of Science and Technology BeijingBeijing 100083China National Engineering Research Center for Advanced Rolling Technology National Engineering Research Center for Flat Rolling EquipmentInstitute of Engin-eering TechnologyUniversity of Science and Technology BeijingBeijing 100083China

Hot deformation of sintered billets by powder metallurgy(PM)is an effective preparation technique for titanium alloys,which is more significant for high-alloying *** this study,Ti–6.5Al–2Zr–Mo–V(TA15)titanium alloy plates were prepared by cold press-ing sintering combined with high-temperature hot *** microstructure and mechanical properties under different process paramet-ers were *** microscope,electron backscatter diffraction,and others were applied to characterize the microstructure evolution and mechanical properties strengthening *** results showed that the chemical compositions were uniformly dif-fused without segregation during sintering,and the closing of the matrix.craters was accelerated by increasing the sintering *** block was hot rolled at 1200℃ with an 80%reduction under only two passes without *** strength and elongation of the plate at 20–25℃ after solution and aging were 1247 MPa and 14.0%,respectively,which were increased by 24.5%and 40.0%,respect-ively,compared with the as-sintered alloy at 1300℃.The microstructure was significantly refined by continuous dynamic recrystalliza-tion,which was completed by the rotation and dislocation absorption of the substructure surrounded by low-angle grain *** hot rolling combined with heat treatment,the strength and plasticity of PM-TA15 were significantly improved,which resulted from the dense,uniform,and fine recrystallization structure and the synergistic effect of multiple slip systems.

关键词： elemental powder powder metallurgy titanium alloy hot rolling strength and plasticity

First-Principles Study of the Influence of Lattice Misfit on the Behavior and the Ductility Effect of Hafnium in Ni-Ni3AI System

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Acta Metallurgica Sinica(English Letters) 2014年第1期27卷 87-94页

作者： Y. Wu j. Guo W. zhang x. Ma Q. zhang j. Hou R. Huang x. Liu Institute of Molecular Engineering and Applied Chemistry Anhui University of Technology Ma'anshan 243002 China Shenyang National Laboratory for Materials Science Instituteof Metal Research Chinese Academy of SciencesShenyang 110016 China College of Physics Science and Technology Shenyang NormalUniversity Huanghe Street 253 Shengyang 110034 China School of Materials Science and Engineering Anhui Universityof Technology Ma'anshan 243002 China

Two Ni/Ni3Al-interface-contained cluster models with/without lattice misfit are studied by first-principles method to clarify the debates about the segregation behaviors of Hafnium （Hf） and ex.lore the influence of lattice misfit on the ductility effect of Hr. It is found that though Hf prefers to substitute A1 rather than Ni in Ni3A1 phase within most of the investigated misfit range, its stronger preferring to Ni phase than NiaA1 phase makes it impossible to go into Ni3A1 phase to occupy A1 site in Ni-Ni3A1 alloys. Bond order analysis in Hf-free case shows that lattice misfit has different effects on the Griffith work of interfacial cleavage 27int/E and the max.mum theoretical shear stress Zmax.of Ni and Ni3A1, contributing to the ex.stence of anomalous strength-temperature phenomena in NiaA1 alloys. However, the addition of Hf will make the 27int/E （or Zmax. of both Ni3A1 and Ni decrease （or increase） with lattice misfit, indicating that the addition of Hf may make the anomalous strength-temperature relationship in Ni3A1 region disappear locally.

关键词： Hafnium Lattice misfit Segregation Ductility

High catalytic performance of neodymium modified Co_(3)O_(4) for toluene ox.dation

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j.urnal of Rare Earths 2024年第1期42卷 94-101,I0004页

作者： Yanqiong Hu Luyue zhang j.anbei zhang Yongj.n Liu School of Architecture and Environment Sichuan UniversityChengdu610065China National Engineering Research Center for Flue Gas Desulfurization Chengdu610065China

Co-based catalysts are the most promising catalysts in catalytic ox.dation of volatile organic compounds(VOCs).Precious metal doping is adopted to improve the catalytic activity of toluene on Co_(3)O_(4) catalysts,but greatly increases its cost along with *** is found that doping a small amount of rare earth(Ce,Pr,Sm and Nd)can dramatically promote the catalytic activity of Co_(3)O_(4).Especially,the Nd-doped Co_(3)O_(4) catalyst ex.ibits ex.ellent catalytic activity with a toluene removal rate of 90% at 162.1℃,which is even better than that of Pt-doped Co_(3)O_(4).Compared with other rare earth metal doping,the Nd doping leads to a higher ratio of Co^(3+)/Co^(2+) and has more ox.gen *** in situ diffuse reflectance infrared Fourier transform spectroscopy(DRIFTS)ex.eriments show that the lattice ox.gen of Nd-Co sample can be utilized at a quite low temperature,while that of pure Co_(3)O_(4) cannot engage in ox.dation reaction when the temperature is below 200℃,which visually demonstrates the main reason for the improved catalytic performance of Nd-Co catalyst.

关键词： Rare earth metals Nd Co_(3)O_(4) Toluene catalytic ox.dation Ox.gen vacancy Lattice ox.gen

Lanthanide-based microlasers:Synthesis,structures,and biomedical applications

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Nano Research 2024年第1期17卷 97-111页

作者： Qian zhang Yawei Liu Kai Liu Hongj.e zhang Engineering Research Center of Advanced Rare Earth Materials(Ministry of Education) Department of ChemistryTsinghua UniversityBeijing 100084China State Key Laboratory of Rare Earth Resource Utilization Changchun Institute of Applied ChemistryChinese Academy of SciencesChangchun 130022China x.angfu Laboratory Jiaxing 314102China

The large size of lasers limits their applications in confined spaces,such as in biosensing and in vivo brain tissue *** this regard,micron-sized lasers have been *** ex.ibit great potential for biological detecting,remote sensing,and depth tracking due to their small sizes,sensitive properties of their spectral fingerprints,and flex.ble positional modulation in the ***-based luminescent materials that possess long ex.ited-state lifetime,narrow emission bandwidth,and upconversion behaviors are promising as gain mediums for novel *** addition,lanthanide-based microlasers could be generated under natural ambient conditions with pumped or continuous light sources,which significantly promotes the practical applications of *** progress in the design,synthesis,and biomedical applications of lanthanide-based microlasers has been outlined in this *** ions doped and upconverted lanthanide-based microlasers are highlighted,which ex.ibit advantageous structures,miniaturized dimensions,and high lasing *** applications of lanthanide-based microlasers are further discussed,the upconverted microlasers show great advantages for biological applications owing to their tunable ex.itation and emission characteristics and ex.ellent environmental ***,perspectives and challenges in the field of lanthanide-based microlasers are presented.

关键词： microlasers lanthanide-based luminescent materials upconversion nanoparticles biological applications