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Whole-exome sequencing identified novel DNAH5 homozygous variants in two consanguineous families with primary ciliary dyskinesia

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中华医学杂志(英文版) 2024年第1期137卷 115-116页

作者： Yang Binyi lei Cheng Xu Yingjie Yang Danhui Lu Chenyang Liu Ying guo Ting Luo Hong Department of Pulmonary and Critical Care Medicine The Second Xiangya Hospital 3. Central South University 4. Changsha 5. Hunan 410011 6. China

Primary ciliary dyskinesia (PCD) is a rare autosomal recessive inherited disease characterized by dysfunction of the motile cilia. DNAH5, the most frequently mutated gene in PCD, encodes a heavy chain of the ciliary outer dynein arms (ODAs). Herein, we report two patients with Kartagener syndrome (sinusitis, bronchiectasis, and situs inversus) in two consanguineous families carrying two novel homozygous DNAH5 variants [Supplementary Figures 1A and 1B, http://***/CM9/B598].

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A lithium–tin fluoride anode enabled by ionic/electronic conductive paths for garnet-based solid-state lithium metal batteries

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Rare Metals 2024年第2期43卷 575-587页

作者： lei Zhang Qian-Kun Meng Xiang-Ping Feng Ming Shen Yu-Qing Zhang Quan-Chao Zhuang Run-guo Zheng Zhi-Yuan wang Yan-Hua Cui Hong-Yu Sun Yan-guo Liu School of Materials Science and Engineering Northeastern UniversityShenyang110819China School of Resources and Materials Northeastern University at QinhuangdaoQinhuangdao066004China School of Materials and Physics China University of Mining&TechnologyXuzhou221116China Institute of Electronic Engineering China Academy of Engineering PhysicsMianyang621900China

The high energy density and stability of solid-state lithium metal batteries(SSLMBs)have garnered great ***-type oxides,especially Li_(6.4)La_(3)Zr_(1.4)Ta_(0.6)O_(12)(LLZTO),with high ionic conductivity,wide electrochemical window,and stability to Li metal anode,are promising solid-state electrolyte(SSEs)materials for ***,Li/LLZTO interface issues including high interface resistance,inhomogeneous Li deposition,and Li dendrite growth have hindered the practical application of ***,a multi-functional Li–SnF_(2) composite anode with Li,LiF,and Li-Sn alloy was specifically designed and *** composite anode improves the wettability to LLZTO,constructing an intimate contact interface between it and ***,ionic/electronic conductive paths in situ formed at the interface can effectively uniform Li deposition and suppress Li *** solid-state symmetric cell exhibits low interface resistance(11Ω·cm^(2)) and high critical current density(1.3 mA·cm^(−2))at 25℃.The full SSLMB based on LiFePO_(4) or LiNi_(0.5)Co_(0.2)Mn_(0.3)O_(2) cathode also shows stable cycling performance and high rate *** work provides a new composite anode strategy for achieving high-energy density and high-safety SSLMBs.

关键词： Solid-state lithium metal batteries(SSLMBs) Lithium-tin fluoride anode Ionic/electronic conductive Interface resistance Lithium dendrite

Breaking the efficiency limitations of dissipative Kerr solitons using nonlinear couplers

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Science China(Physics,Mechanics & Astronomy) 2024年第3期67卷 88-94页

作者： Ming Li Xiao-Xiao Xue Yan-lei Zhang Xin-Biao Xu Chun-Hua Dong Guang-Can guo Chang-Ling Zou CAS Key Laboratory of Quantum Information University of Science and Technology of ChinaHefei 230026China CAS Centerfor Excellence in Quantum Information and Quantum Physics University of Science and Technology of ChinaHefei 230026China Department of Electronic Engineering Beijing National Research Center for Information Science and TechnologyTsinghua University Beijing 100084China

Dissipative Kerr solitons(DKS) have long been suffering from poor power conversion efficiency when driven by continuous-wave lasers. By deriving the critical coupling condition of a multimode nonlinear optics system in a generalized theoretical framework,two efficiency limitations of the conventional pump method of DKS are revealed: the effective coupling rate is too small and is also power-dependent. A general approach is provided to resolve this challenge by introducing two types of nonlinear couplers to couple the soliton cavity and CW input through nonlinear processes. The collective coupler opens multiple coupling channels and the self-adaptive coupler builds a power-independent effective external coupling rate to the DKS for approaching the generalized critical coupling condition, which promises near-unity power conversion efficiencies. For instance, a conversion efficiency exceeding 90% is predicted for aluminum nitride microrings with a nonlinear coupler utilizing second-harmonic generation. The mechanism applies to various nonlinear processes, including Raman and Brillouin scattering, and thus paves the way for micro-solitons toward practical applications.

关键词： Kerr frequency comb conversion efficiency nonlinear coupler

Manipulation of low-refractive-index particles using customized dark traps

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Photonics Research 2024年第6期12卷 1334-1343页

作者： MINRU HE YANSHENG LIANG XUE YUN SHAOWEI waNG TIANYU ZHAO LINQUAN guo XINYU ZHANG SHIQI KUANG JINXIAO CHEN MING lei MOE Key Laboratory for Non-Equilibrium Synthesis and Modulation of Condensed Matter School of PhysicsXi’an Jiaotong UniversityXi’an 710049China State Key Laboratory of Electrical Insulation and Power Equipment Xi’an Jiaotong UniversityXi’an 710049China

Low-refractive-index particles play significant roles in physics,drug delivery,biomedical science,and other ***,they have not attained sufficient utilization in active manipulation due to the repulsive effect of *** this work,the establishment of customized dark traps is demonstrated to fulfill the demands of versatile manipulation of low-refractive-index *** customized dark traps are generated by assembling generalized perfect optical vortices based on the free lens modulation method,by which the beams’shape,intensity,and position can be elaborately designed with size independent of topological *** the customized dark traps with high quality and high efficiency,rotation along arbitrary trajectories with controllable speed,parallel manipulation,and sorting of low-refractive-index particles by size can be *** unprecedented flexibility and quality,the customized dark traps provide tremendous potential in optical trapping,lithography,and biomedicine.

关键词： refractive rotation versatile

Wide Stopband Substrate Integrated waveguide Filter Using Bisection and Trisection Coupling in Multilayer

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Chinese Journal of Electronics 2024年第2期33卷 436-442页

作者： Peng CHU Jianguo FENG Peng ZHU lei guo Long ZHANG leilei LIU guoqing LUO Ke WU College of Electronic and Optical Engineering Nanjing University of Posts and Telecommunications National and Local Joint Engineering Laboratory of RF Integration and Micro-Assembly Technology School of Information and Communication Engineering Dalian University of Technology Guangdong Provincial Key Laboratory of Optoelectronic Information Processing Chips and Systems Sun Yat-sen University College of Electronics and Information Engineering Shenzhen University School of Electronics and Information Hangzhou Dianzi University Poly-Grames Research Center Polytechnique Montreal

This article presents a highly efficient method for substrate-integrated-waveguide（SIW） filters to achieve very wide stopbands. By employing the proposed trisection slots in addition to the bisection slots as the inter-coupling structures, all spurious modes below TE505 of a SIW filter working in the fundamental mode TE101（f0）can be eliminated without requiring additional structure or complex theoretical analysis, without affecting the design of the fundamental passbands, and without degrading the performance of the filters. For verification, two prototype filters are designed, fabricated, and measured with wide stopbands up to 4.15f0 and 4.83f0, respectively. The proposed technique could facilitate the development of high-performance wide-stopband SIW filters for microwave/wireless circuits and systems.

关键词： Microwave measurement Couplings Filters Prototypes Nonhomogeneous media Microwave theory and techniques Microwave circuits

Atroposelective Synthesis of 3-Aryl-Indoles Through a One-Pot 2,3-Difunctionalization of Simple Indoles

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CCS Chemistry 2024年第7期6卷 1672-1680页

作者： lei Dai Yu-Li Sun Jiami guo Xueting Zhou Qingqin Huang Yixin Lu Department of Chemistry National University of SingaporeSingapore 117543 Joint School of National University of Singapore and Tianjin University International Campus of Tianjin UniversityBinhai New CityFuzhouFujian350207

Indole and its derivatives represent the most important heterocycles that are widely present in bioactive molecules,natural products and advanced materials,and thus functionalization of simple indoles to construct complex indole derivatives is a research area of great current interest.2,3-Difunctionalization of indoles has been extensively studied,but the reported examples are limited to the synthesis of 2,3-disubstituted indole derivatives or dearomatized products containing central *** now,atroposelective 2,3-difunctionalization of simple indoles for the synthesis of axially chiral molecules is *** this article,we report a straightforward and general strategy for atroposelective 2,3-difunctionalization of simple indoles,forming indole-containing axially chiral products in good yields and excellent *** strategy we introduce herein may lead to the discovery of new approaches for multifunctionalization of indoles and other heterocyclic scaffolds,thus accessing novel axially chiral heteroarene-containing scaffolds that may find applications in medicinal chemistry and asymmetric catalysis.

关键词： axial chirality atroposelectivity sequential catalysis difunctionalization of indoles chiral phosphoric acid

Constructing oxygen deficiency-rich V_(2)O_(3)@PEDOT cathode for high-performance aqueous zinc-ion batteries

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Rare Metals 2024年第2期43卷 635-646页

作者： Dong-Fei Sun Zi-Juan wang Tian Tian Xin Yu Dan-Dan Yu Xiao-Zhong Zhou guo-Fu Ma Zi-Qiang lei Key Laboratory of Eco-functional Polymer Materials of the Ministry of Education Key Laboratory of Eco-environmental Polymer Materials of Gansu ProvinceSchool of College of Chemistry and Chemical EngineeringNorthwest Normal UniversityLanzhou730070China

Aqueous zinc-ion batteries(AZIBs)have attracted widespread attention due to the advantages of high safety and environmental *** V_(2)O_(3) is a promising cathode,the strong electrostatic interaction between Zn^(2+) and V_(2)O_(3) crystal,and the sluggish reaction kinetics still limit their application in ***,the oxygen defects rich V_(2)O_(3) with conducive poly(3,4-ethylenedioxythiophene)(PEDOT)shell(V_(2)O_(3)-Od@PEDOT)was fabricated for AZIBs by combining the sulfur-assisted thermal reduction and in-situ polymerization *** introduced oxygen vacancies of V_(2)O_(3)–Od@PEDOT weaken the electrostatic interaction between Zn^(2+) and the host material,improving the interfacial electron transport,while the PEDOT coating enhances the structural stability and conductivity of V_(2)O_(3),thus accelerating the reaction *** on the advantages,V_(2)O_(3)–Od@PEDOT electrode delivers a reversible capacity of 495 mAh·g^(−1) at 0.1 A·g^(−1),good rate capability(189 mAh·g^(−1)at 8.0 A·g^(−1)),and an impressive cycling stability with 90.1%capacity retention over 1000 cycles at 8.0 A·g^(−1).The strategy may provide a path for exploiting the other materials for high performance AZIBs.

关键词： Oxygen defects V_(2)O_(3) PEDOT Electrochemical self-optimization Aqueous zinc-ion batteries

ATP6AP2,a regulator of LRP6/β-catenin protein trafficking,promotes Wnt/β-catenin signaling and bone formation in a cell type dependent manner

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Bone Research 2024年第2期12卷 453-468页

作者： lei Xiong Hao-Han guo Jin-Xiu Pan Xiao Ren Daehoon Lee Li Chen Lin Mei Wen-Cheng Xiong Department of Neurosciences School of MedicineCase Western Reserve UniversityClevelandOH 44106USA Louis Stoke VA Medical Center ClevelandOH 44106USA

Wnt/β-catenin signaling is critical for various cellular processes in multiple cell types,including osteoblast(OB)differentiation and *** how Wnt/β-catenin signaling is regulated in OBs remain ***6AP2,an accessory subunit of V-ATPase,plays important roles in multiple cell types/organs and multiple signaling ***,little is known whether and how ATP6AP2 in OBs regulates Wnt/β-catenin signaling and bone *** we provide evidence for ATP6AP2 in the OB-lineage cells to promote OB-mediated bone formation and bone homeostasis selectively in the trabecular bone *** knocking out(CKO)ATP6AP2 in the OB-lineage cells(Atp6ap2^(Ocn-Cre))reduced trabecular,but not cortical,bone formation and bone *** and cellular biochemical studies revealed that LRP6 and N-cadherin were reduced in ATP6AP2-KO BMSCs and OBs,but not *** in vitro and in vivo studies revealed impairedβ-catenin signaling in ATP6AP2-KO BMSCs and OBs,but not osteocytes,under both basal and Wnt stimulated conditions,although LRP5 was decreased in ATP6AP2-KO osteocytes,but not *** cell biological studies uncovered that osteoblastic ATP6AP2 is not required for Wnt3a suppression ofβ-catenin phosphorylation,but necessary for LRP6/β-catenin and N-cadherin/β-catenin protein complex distribution at the cell membrane,thus preventing their *** of activeβ-catenin diminished the OB differentiation deficit in ATP6AP2-KO *** together,these results support the view for ATP6AP2 as a critical regulator of both LRP6 and N-cadherin protein trafficking and stability,and thus regulatingβ-catenin levels,demonstrating an un-recognized function of osteoblastic ATP6AP2 in promoting Wnt/LRP6/β-catenin signaling and trabecular bone formation.

关键词： LRP6 impaired organs

Fusion of a rice endogenous N-methylpurine DNA glycosylase to a plant adenine base transition editor ABE8e enables A-toK base editing in rice plants

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aBIOTECH 2024年第2期5卷 127-139页

作者： Yucai Li Shaoya Li Chenfei Li Chen Zhang lei Yan Jingying Li Yubing He Yan guo Lanqin Xia Institute of Crop Sciences(ICS) Chinese Academy of Agricultural Sciences(CAAS)Beijing 100081China State Key Laboratory of Plant Physiology and Biochemistry College of Biological SciencesChina Agricultural UniversityBeijing 100193China Hainan Yazhou Bay Seed Laboratory/National Nanfan Research Institute(Sanya) CAASSanya 572024China

Engineering of a new type of plant base editor for simultaneous adenine transition and transversion within the editing window will greatly expand the scope and potential of base editing in directed evolution and crop ***,we isolated a rice endogenous hypoxanthine excision protein,N-methylpurine DNA glycosylase(OsMPG),and engineered two plant A-to-K(K=G or T)base editors,rAKBE01 and rAKBE02,for simultaneous adenine transition and transversion base editing in rice by fusing OsMPG or its mutant mOsMPG to a plant adenine transition base editor,*** further coupled either OsMPG or mOsMPG with a transactivation factor VP64 to generate rAKBE03 and rAKBE04,*** these four rAKBEs,at five endogenous loci in rice protoplasts,indicated that rAKBE03 and rAKBE04 enabled higher levels of A-to-G base transitions when compared to ABE8e and ***,whereas rAKBE01 only enabled A-to-C/T editing at one endogenous locus,in comparison with rAKBE02 and rAKBE03,rAKBE04 could significantly improve the A-to-C/T base transversion efficiencies by up to 6.57-and 1.75-fold in the rice protoplasts,***,although no stable lines with A-to-C transversion were induced by rAKBE01 and rAKBE04,rAKBE04 could enable simultaneous A-to-G and A-to-T transition and transversion base editing,at all the five target loci,with the efficiencies of A-to-G transition and A-to-T transversion editing ranging from 70.97 to 92.31%and 1.67 to 4.84%in rice stable lines,***,these rAKBEs enable different portfolios of editing products and,thus,now expands the potential of base editing in diverse application scenario for crop improvement.

关键词： Rice(Oryza sativa L) Rice N-methylpurine DNA glycosylase(OsMPG) A-to-K base editor(AKBE) Transactivation module VP64