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| 1 | Clinical classification and gene mutation of Chinese probands with Charcot-Marie-Tooth disease Analysis of 57 cases显示文摘Charcot-Marie-Tooth(CMT) disease is the most common inherited peripheral neuropathic disorder.CMT is clinically and genetically heterogeneous.To date,27 genes associated with the disease have been cloned.The present study carried out clinical classification according to clinical,electrophysiological and pathological features,conducted inheritance classification according to inheritance patterns,and performed mutation analysis of 13 CMT disease genes(PMP22,CX32,HSPB1,MNF2,MPZ,HSPB8,GDAP1,NFL,EGR2,SIMPLE,RAB7,LMNA,MTMR2) in 57 Chinese probands with CMT.Five cases of AD-CMT1 and 13 cases of sporadic CMT1 were diagnosed as CMT1A;five cases of X-CMT1,one case of X-CMT2 and one case of sporadic CMT1 were diagnosed as CMTX1;four cases of AD-CMT2 were diagnosed as CMT2F;one case of AD-CMT2 and one case of sporadic CMT2 were diagnosed as CMT2A2;one case of AD-CMT2 was diagnosed as CMT2L;one case of AD-CMT2 was diagnosed as CMT2J;one case of AR-CMT1 was diagnosed as CMT4A.Among the 57 CMT probands,seven genotypes were determined among 34 patients,with a detection rate of 59.6%.The results indicated that the clinical classification and inheritance classification are indispensable for selecting potential disease genes for mutation detection,and for efficient molecular diagnosis. | Ruxu Zhang Xiaobo Li Xiaohong Zi Shunxiang Huang Fufeng Zhang Kun Xia Qian Pan Beisha Tang | 2011 | Neural Regeneration Research2011，6，9： | 4 |
| 2 | Central Pattern Generator(CPG)Control of a Biomimetic Robot Fish for Multimodal Swimming显示文摘This paper introduces the design and control of a biomimetic robot fish for multimodal swimming.The biomimetic design consists of three parts:the rigid head,the wire-driven body and the compliant tail.The control is an improved Central Pattern Generator(CPG)with the high-level control command:(M,co,B,R),where M is the amplitude,co is the angular velocity,B is the offtet and R is the time ratio between two phases forming one flapping cycle.This method differs from previous research in two aspects:(1)The CPG control is firstly implemented on the wire-driven robot fish.(2)The improved CPG model synthesizes symmetrical flapping in cruising and asymmetrical flapping in turning for the robot fish.The asymmetrical flapping refers to the asymmetry of the offset and the time ratio.This combination of the design and the control has several advantages over the existing multimodal swimming robot fishes.First,it uses just one driving motor for undulatory oscillation while the others need to use two or more motors.Second,with just one motor,the CPG control can be easily implemented.Third,the use of the time ratio,R,makes the robot fish turn more naturally and effectively.Experimental results show the robot fish achieved the maximum speed of 1.37 Body Length/Second(BL.s-1)and the largest turning rate of 4577s.Additionally,in many swimming conditions,its Strouhal Number falls in the range from 0.2 to 0.4,which implies the robot fish is efficient. | Fengran Xie Yong Zhong Ruxu Du Zheng Li | 2019 | Journal of Bionic Engineering2019，16，2： | 4 |
| 3 | Numerical simulation of the effect of void fraction and inlet velocity on two-phase turbulence in bubble-liquid flows显示文摘有水泡是否提高或减少液体骚乱的矛盾的意见。在这篇论文,空部分的效果和水泡液体上的入口速度,在一条二维的隧道的多重水泡液体喷气的二阶段的骚乱被使用二阶段的秒顺序时刻骚乱模型学习。结果证实在实验观察并且在参考书报导在低空部分和低入口速度,水泡提高液体骚乱的现象,而在高空的部分和高入口速度水泡减少液体骚乱。 | Lixing Zhou Rongxian Li Ruxu Du | 2006 | Acta Mechanica Sinica2006，22，5： | 3 |
| 4 | Oral remdesivir derivative VV116 is a potent inhibitor of respiratory syncytial virus with efficacy in mouse model显示文摘Dear Editor,Respiratory syncytial virus(RSV)is the leading cause of serious lower respiratory tract disease in children under 5 years of age worldwide,causing an estimated 3.2 million hospitalizations and 120,000 deaths in children globally per year.Furthermore,nearly all children can be infected with RSV by 2 years of age,and individuals can be repeatedly re-infected with RSV throughout life,which poses great threats to infants,the elderly. | Ruxue Zhang Yumin Zhang Wei Zheng Weijuan Shang Yan Wu Ning Li Jun Xiong Hualiang Jiang Jingshan Shen Gengfu Xiao Yuanchao Xie Leike Zhang | 2022 | Signal Transduction and Targeted Therapy2022，7，5： | 2 |
| 5 | Medical Additive Manufacturing: From a Frontier Technology to the Research and Development of Products显示文摘1.Research and development(R&D)and the challenges of raw materials for medical additive manufacturing Raw materials for medical additive manufacturing have a wide range of commonalities that are also seen in many other fields,making them an important basis in the field of three-dimensional(3D)printing.Problems and challenges related to material types,powder properties,formability,viscoelasticity,and so forth also share common features.For example,many metal materials are used in the field of aviation,while metals,polymers,and inorganic materials are used in the field of biomedicine.The most widely used materials in biomedicine are biocompatible.Various homogeneous and non-homogeneous composites are also available for 3D printing,and impose an additional challenge in additive manufacturing;the use of heterogeneous composites in 3D printing is particularly challenging. | Guixing Qiu Wenjiang Ding Wei Tian Ling Qin Yu Zhao Lianmeng Zhang Jian Lu Daijie Chen Guangyi Yuan Chengtie Wu Bingheng Lu Ruxu Du Jimin Chen Mo Elbestawi Zhongwei Gu Dichen Li Wei Sun Yuanjin Zhao Jie He Dadi Jin Bin Liu Kai Zhang Jianmo Li Kam WLeong Dewei Zhao Dingjun Hao Yingfang Ao Xuliang Deng Huilin Yang ShaoKeh Hsu Yingqi Chen Long Li Jianping Fan Guohui Nie Yun Chen Hui Zeng Wei Chen Yuxiao Lai | 2020 | Engineering2020，6，11： | 1 |
| 6 | Development of a validated HPLC-PAD-APCI/MS method for the identification and determination of iridoid glycosides in Lamiophlomis rotate显示文摘 | Li Maoxing Zhang Ruxue Li Caixia | 2010 | Anal Methods2010，2，： | 1 |
| 7 | Rehmannia glutinosa: review of botany,chemistry and pharmacology显示文摘 | Zhang RuXue Li MaoXing Jia ZhengPing | 2008 | J Ethnopharmacol2008，117，2： | 1 |
| 8 | Influence of mixed organic cations on the nonlinear optical properties of lead tri-iodide perovskites显示文摘Metal halide perovskite materials have been widely studied recently due to their excellent optoelectronic properties.Among these materials,organic-inorganic hybrid perovskites have attracted much attention because of their relatively soft framework,which makes them more suitable for nonlinear optical(NLO)applications.However,there is rare physical mechanism study on the coexistence of two-photon absorption(TPA)and saturable absorption(SA)in organic-inorganic hybrid perovskite materials.To clarify this issue,the NLO properties of mixed cation perovskite MA1-xFAxPbI3[MA=CH3NH3,FA=CH(NH2)2,x=0,0.2,0.4,0.6,and 0.8]thin films are investigated in this paper.Based on the nonlinear transmittance and femtosecond-transient absorption spectrum measurements,it is found that the MA1-xFAxPbI3 materials exhibit NLO behavior dependent on excitation intensity.The TPA coefficient of MA1-xFAxPbI3 decreases with the increase of formamidinium(FA)content,while the relevant saturable intensity increases.In addition,it is revealed that the linear absorption process from valence band 2 to valance band 1 still exists even under a very low excitation intensity.With the increase of excitation intensity,the light transmittance at 1300 nm decreases first and then increases sharply,which also supports the explanation for the coexistence of TPA and SA.It is expected that our findings will promote the application of perovskite materials in nonlinear optoelectronic devices. | XUANYU ZHANG SHUYU XIAO RUXUE LI TINGCHAO HE GUICHUAN XING AND RUI CHEN | 2020 | Photonics Research2020，8，9： | 1 |
| 9 | Ameliorating effect and potential mechanism of Rehmannia glutinosa oligosaccharides on the impaired glucose metabolism in chronic stress rats fed with high-fat diet显示文摘 | Ruxue Zhang Jun Zhou Maoxing Li Haigang Ma Jianguo Qiu Xiaohong Luo Zhengping Jia | 2013 | Phytomedicine2013，，： | 1 |
| 10 | Alkali-soluble polysaccharide, isolated from Lentinus edodes, induces apoptosis and G2/M cell cycle arrest in H22 cells through microtubule depolymerization显示文摘 | YOU Ruxu LIU Jinyu LI Shijun | 2014 | Phytotherapy Research2014，28，12： | 1 |
| 11 | A novel transgenic mouse model of Chinese CharcotMarie-Tooth disease type 2L显示文摘We previously found that the K141N mutation in heat shock protein B8(HSPB8) was responsible for Charcot-Marie-Tooth disease type 2L in a large Chinese family.The objective of the present study was to generate a transgenic mouse model bearing the K141N mutation in the human HSPB8 gene,and to determine whether this K141NHSPB8 transgenic mouse model would manifest the clinical phenotype of Charcot-Marie-Tooth disease type 2L,and consequently be suitable for use in studies of disease pathogenesis.Transgenic mice overexpressing K141NHSPB8 were generated using K141N mutant HSPB8 cDNA cloned into a pCAGGS plasmid driven by a human cytomegalovirus expression system.PCR and western blot analysis confirmed integration of the K141NHSPB8 gene and widespread expression in tissues of the transgenic mice.The K141NHSPB8 transgenic mice exhibited decreased muscle strength in the hind limbs and impaired motor coordination,but no obvious sensory disturbance at 6 months of age by behavioral assessment.Electrophysiological analysis showed that the compound motor action potential amplitude in the sciatic nerve was significantly decreased,but motor nerve conduction velocity remained normal at 6 months of age.Pathological analysis of the sciatic nerve showed reduced myelinated fiber density,notable axonal edema and vacuolar degeneration in K141NHSPB8 transgenic mice,suggesting axonal involvement in the peripheral nerve damage in these animals.These findings indicate that the K141NHSPB8 transgenic mouse successfully models Charcot-Marie-Tooth disease type 2L and can be used to study the pathogenesis of the disease. | Ruxu Zhang Fufeng Zhang Xiaobo Li Shunxiang Huang Xiaohong Zi Ting Liu Sanmei Liu Xuning Li Kun Xia Qian Pan Beisha Tang | 2014 | Neural Regeneration Research2014，9，4： | 1 |
| 12 | Recommendations for the diagnosis and treatment of paroxysmal kinesigemc dyskinesia: an expert consensus in China显示文摘Paroxysmal dyskinesias are a group of neurological diseases characterized by intermittent episodes of involuntary movements with different causes.Paroxysmal kinesigenic dyskinesia(PKD)is the most common type of paroxysmal dyskinesia and can be divided into primary and secondary types based on the etiology.Clinically,PKD is characterized by recurrent and transient attacks of involuntary movements precipitated by a sudden voluntary action.The major cause of primary PKD is genetic abnormalities,and the inheritance pattern of PKD is mainly autosomal-dominant with incomplete penetrance.The proline-rich transmembrane protein 2(PRRT2)was the first identified causative gene of PKD,accounting for the majority of PKD cases worldwide.An increasing number of studies has revealed the clinical and genetic characteristics,as well as the underlying mechanisms of PKD.By seeking the views of domestic experts,we propose an expert consensus regarding the diagnosis and treatment of PKD to help establish standardized clinical evaluation and therapies for PKD.In this consensus,we review the clinical manifestations,etiology,clinical diagnostic criteria and therapeutic recommendations for PKD,and results of genetic analyses in PKD patients performed in domestic hospitals. | Li Cao Xiaojun Huang Ning Wang Zhiying Wu Cheng Zhang Weihong Gu Shuyan Cong Jianhua Ma Ling Wei Yanchun Deng Qi Fang Qi Niu Jin Wang Zhaoxia Wang You Yin Jinyong Tian Shufen Tian Hongyan Bi Hong Jiang Xiaorong Liu Yang Lu Meizhen Sun Jianjun Wu Erhe Xu Tao Chen Tao Chen Xu Chen Wei Li Shujian Li Qinghua Li Xiaonan Song Ying Tang Ping Yang Yun Yang Min Zhang Xiong Zhang Yuhu Zhang Ruxu Zhang Yi Ouyang Jintai Yu Quanzhong Hu Qing Ke Yuanrong Yao Zhe Zhao Xiuhe Zhao Guohua Zhao Furu Liang Nan Cheng Jianhong Han Rong Peng Shengdi Chen Beisha Tang | 2021 | Translational Neurodegeneration2021，10，1： | 1 |
| 13 | Heterogeneously integrated quantum-dot emitters efficiently driven by a quasi-BIC-supporting dielectric nanoresonator显示文摘Bound states in the continuum(BICs) can make subwavelength dielectric resonators sustain low radiation leakage, paving a new way to minimize the device size, enhance photoluminescence, and even realize lasing. Here, we present a quasi-BIC-supporting Ga As nanodisk with embedded In As quantum dots as a compact bright on-chip light source, which is realized by heterogeneous integration, avoiding complex multilayered construction and subsequent mismatch and defects. The emitters are grown inside the nanodisk to match the mode field distribution to form strong light–matter interaction. One fabricated sample demonstrates a photoluminescence peak sustaining a quality factor up to 68 enhanced by the quasi-BIC, and the emitting effect can be further promoted by improving the epilayer quality and optimizing the layer-transferring process in the fabrication. This work provides a promising solution to building an ultracompact optical source to be integrated on a silicon photonic chip for high-density integration. | LI LIU RUXUE WANG XUYI ZHAO WENFU YU YI JIN QIAN GONG AIMIN WU | 2022 | Photonics Research2022，10，8： | 1 |
| 14 | Rehmannia glutinosa : Review of botany, chemistry and pharmacology 显示文摘 | Zhang Ruxue Li Maoxing Ji Zhengping | 2008 | J Ethnophar- macol2008，117，2： | 1 |
| 15 | A novel image-fusion method based on the un-mixing of mixed MS sub-pixels regarding high-resolution DSM显示文摘A major reason for the spectral distortions of fused images generated by current image-fusion methods is that the fused versions of mixed multispectral(MS)sub-pixels(MSPs)corresponding to panchromatic(PAN)pure pixels remain mixed.The MSPs can be un-mixed spectrally to pure pixels having the same land cover classes in a fine classification map during the fusion process.Since it is difficult to produce such a land cover classification map using only MS and PAN images,a Digital Surface Model(DSM)derived from airborne Light Detection And Ranging data were employed in this study to facilitate the classification.In a novel fusion method proposed in this paper,MSPs near and across boundaries between vegetation and non-vegetation are identified using MS,PAN,and normalized Digital Surface Model(nDSM).The identified MSPs then are fused to pure pixels with respect to the corresponding land cover class in the classification map.In a test on WorldView-2 images over an urban area and the corresponding nDSM,the fused image generated by the proposed method was visually and quantitatively compared with fused images obtained using common image-fusion methods.The fused images generated by the proposed method yielded minimal spectral distortions and sharpened boundaries between vegetation and non-vegetation. | Hui Li Linhai Jing Zhongchang Sun Junjie Li RuXu Yunwei Tang Fulong Chen | 2016 | International Journal of Digital Earth2016，9，6： | 0 |
| 16 | Coordinatively unsaturated cobalt single-atom nanozymes for visual pesticides detection by smartphone-based platform显示文摘By adjusting the coordination environment of single-atom catalysts,the enzyme-like activity can be finely tuned for highly sensitive biosensing.Herein,we demonstrated that coordinatively unsaturated cobalt-nitrogen sites doped within porous carbon(SA-CoN_(3))could serve as highly efficient oxidase mimic.Compared with the typical planar four-coordination structure(SA-CoN_(4)),the as-obtained single-atom Co nanozymes anchored by three nitrogen atoms are found to display much higher oxidase-like catalytic efficiency.Combined theoretical and experimental analysis revealed that the coordinatively unsaturated Co sites could facilitate adsorption and activation of O_(2) molecule and thus improve their oxidase-like activity.Based on the enhanced oxidase-like activity of SA-CoN_(3),a paper/smartphone sensor for organophosphorus pesticides(OPs)was successfully constructed and used to quantify glyphosate in environmental and food samples with a low detection limit of 0.66μM.This work not only highlights the important role of coordination unsaturation of SA nanozymes for promoting oxidase-like activity,but also provides an easy and cost-effective way to conduct effective quantification of OPs in the field. | Fangning Liu Zhe Li Hengya Wei Peng Xu Ge Kang Shicheng Zhu Tingting Wang Ruxue He Chuanxia Chen Yizhong Lu | 2024 | Nano Research2024，17，4： | 0 |
| 17 | Coal Rock Condition Detection Model Using Acoustic Emission and Light Gradient Boosting Machine显示文摘Coal rock mass instability fracture may result in serious hazards to underground coal mining.Acoustic emissions(AE)stimulated by internal structure fracture should carry lots of favorable information about health condition of rock mass.AE as a sensitive non-destructive test method is gradually utilized to detect anomaly conditions of coal rock.This paper proposes an improved multi-resolution feature to extract AE waveform at different frequency resolutions using Coilflet Wavelet Transform method(CWT).It is further adopt an efficient Light Gradient Boosting Machine(LightGBM)by several cascaded sub weak classifier models to merge AE features at different views of frequency for coal rock anomaly damage recognition.The results denote that the proposed method achieves excellent recognition performance on anomaly damage levels of coal rock.It is an effective method to detect the critical stability further to predict the rock mass bursting in time. | Jing Li Yong Yang Hongmei Ge Li Zhao Ruxue Guo | 2020 | Computers, Materials & Continua2020，，4： | 0 |
| 18 | Rapid genetic screening of Charcot-Marie-Tooth disease type 1A and hereditary neuropathy with liability to pressure palsies patients显示文摘We used the allele-specific PCR-double digestion method on peripheral myelin protein 22 (PMP22) to determine duplication and deletion mutations in the proband and family members of one family with Charcot-Marie-Tooth disease type 1 and one family with hereditary neuropathy with liability to pressure palsies. The proband and one subclinical family member from the Charcot-Marie-Tooth disease type 1 family had a PMP22 gene duplication; one patient from the hereditary neuropathy with liability to pressure palsies family had a PMP22 gene deletion. Electron microscopic analysis of ultrathin sections of the superficial peroneal nerve from the two probands demonstrated demyelination and myelin sheath hyperplasia, as well as an 'onion-like' structure in the Charcot-Marie-Tooth disease type 1A patient. We observed an irregular thickened myelin sheath and 'mouse-nibbled'-like changes in the patient with hereditary neuropathy with liability to pressure palsies. In the Charcot-Marie-Tooth disease type 1A patient, nerve electrophysiological examination revealed moderate-to-severe reductions in the motor and sensory conduction velocities of the bilateral median nerve, ulnar nerve, tibial nerve, and sural nerve. Moreover, the compound muscle action potential amplitude was decreased. In the patient with hereditary neuropathy with liability to pressure palsies, the nerve conduction velocity of the bilateral tibial nerve and sural nerve was moderately reduced, and the nerve conduction velocity of the median nerve and ulnar nerve of both upper extremities was slightly reduced. | Xiaobo Li Xiaohong Zi Lin Li Yajing Zhan Shunxiang Huang Jin Li Xuning Li Xigui Li Zhengmao Hu Kun Xia Beisha Tang Ruxu Zhang | 2012 | Neural Regeneration Research2012，7，32： | 0 |
| 19 | Energy-saving windows derived from transparent显示文摘Energy-saving windows play a crucial role in sustainable development of green buildings.Integrating super-insulating aerogels with glasses is an attractive method to minimize the energy loss through building windows.However,achieving energy-saving windows with high transparency,super insulation,mechanical robustness,low cost,and scalable aerogels remains a challenge.In a recent study published in Nature Energy,Smalyukh and coworkers synthesized a highly transparent silanized cellulose aerogel for energy-saving windows,overcoming the challenges.This work promotes the practical application of aerogel-based glazing and provides an effective way to save energy of buildings. | Ruxue Du Siqi Wang Tingxian Li | 2024 | Nano Research Energy2024，3，1： | 0 |
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