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| 1 | Exome sequencing confirms molecular diagnoses in 38 Chinese families with hereditary spherocytosis显示文摘Hereditary spherocytosis(HS), the most common cause of congenital hemolytic anemia, is caused by deficiency of the erythrocyte membrane proteins. Five causative genes(ANK1, SPTB, SPTA1, SLC4 A1, and EPB42) have been identified. To date,molecular genetic studies have been performed in different populations, including the American, European, Brazilian, Japanese and Korean populations, whereas only a few studies have been described in the Chinese population. Here, by reanalysis of the exome data, we revealed causative mutations and established a definitive diagnosis of HS in all 38 Chinese families. We found 34 novel mutations and four reported mutations in three known HS-causing genes—17 in ANK1, 17 in SPTB and four in SLC4 A1,suggesting that ANK1 and SPTB are the major genes in Chinese patients with HS. All of the ANK1 or SPTB mutations, scattered throughout the entire genes, are non-recurrent; and most of them are null mutations, which might cause HS via a haploinsufficiency mechanism. De novo mutations in ANK1 or SPTB often occur with an unexpected high frequency(87.5% and64.2%, respectively). Our study updates our knowledge about the genetic profile of HS in Chinese and shows that family-based,especially parent-offspring trio, sequencing analysis can help to increase the diagnostic power and improve diagnostic efficiency. | Rongrong Wang Shuanghao Yang Ming Xu Jia Huang Hongyan Liu Weiyue Gu Xue Zhang | 2018 | Science China(Life Sciences)2018，61，8： | 22 |
| 2 | Research on wind-induced responses of a large-scale membrane structure显示文摘The wind-induced responses of a large-scale membrane structure, Expo Boulevard, are evaluated in this study. To obtain the wind pressure distribution on the roof surface, a wind tunnel test is performed. A brief analysis of wind pressure on the membrane roof is conducted first and then an analysis of the wind-induced responses of the structure is carried out using a numerical integral method in the time domain. In the process of calculation, the geometrical nonlinearity is taken into account. Results indicate that mean, RSM and peak values of the structure responses increase nonlinearly while the approaching flow velocity increases. Strong nonlinear characteristics are observed in the displacement responses, whereas the responses of nodal stress and cable axial force show minimal nonlinear properties when the membrane structure is subjected to wind loads. Different values of the damping ratio only have a minimal impact on the RSM response of the structure because the background component is a dominant part of the total dynamic response and the resonant component is too small. As the damping ratio increases from 0.02 to 0.05, the RMS responses of vertical displacement, nodal stress and cable axial force decrease by 8.1%, 6.7% and 17.9%, respectively. Since the mean component plays a significant role in the wind-induced response, the values of the gust response factor are not high for Expo Boulevard. | Zhou Xuanyi Han Zhihui Gu Ming Zhang An-an Zhang Weiyu Fang Wei | 2013 | Earthquake Engineering and Engineering Vibration2013，12，2： | 4 |
| 3 | Tuning Atomically Dispersed Fe Sites in Metal–Organic Frameworks Boosts Peroxidase‑Like Activity for Sensitive Biosensing显示文摘Although nanozymes have been widely developed,accurate design of highly active sites at the atomic level to mimic the electronic and geometrical structure of enzymes and the exploration of underlying mechanisms still face significant challenges.Herein,two functional groups with opposite electron modulation abilities(nitro and amino)were introduced into the metal–organic frameworks(MIL-101(Fe))to tune the atomically dispersed metal sites and thus regulate the enzymelike activity.Notably,the functionalization of nitro can enhance the peroxidase(POD)-like activity of MIL-101(Fe),while the amino is poles apart.Theoretical calculations demonstrate that the introduction of nitro can not only regulate the geometry of adsorbed intermediates but also improve the electronic structure of metal active sites.Benefiting from both geometric and electronic effects,the nitro-functionalized MIL-101(Fe)with a low reaction energy barrier for the HO*formation exhibits a superior POD-like activity.As a concept of the application,a nitro-functionalized MIL-101(Fe)-based biosensor was elaborately applied for the sensitive detection of acetylcholinesterase activity in the range of 0.2–50 mU mL−1 with a limit of detection of 0.14 mU mL−1.Moreover,the detection of organophosphorus pesticides was also achieved.This work not only opens up new prospects for the rational design of highly active nanozymes at the atomic scale but also enhances the performance of nanozyme-based biosensors. | Weiqing Xu Yikun Kang Lei Jiao Yu Wu Hongye Yan Jinli Li Wenling Gu Weiyu Song Chengzhou Zhu | 2020 | Nano-Micro Letters2020，12，12： | 4 |
| 4 | LHCGR and ALMS1 defects likely cooperate in the development of polycystic ovary syndrome indicated by double-mutant mice显示文摘Polycystic ovary syndrome(PCOS)is a heterogeneous disorder with evidence of polygenetic components,and obesity may be a risk factor for hyperandrogen ism.Previous studies have shown that LHCGR is en riched in the ovary and LHCGR deficiency causes infertility without typical PCOS phenotypes.ALMS1 is implicated in obesity and hyperandrogenism,the common phenotypes among PCOS patients.Through whole-exome sequencing of 22 PCOS families and targeted candidate gene sequencing of additional 65 sporadic PCOS patients,we identified potential causative mutations in LHCGR and ALMS1 in a sibling-pair PCOS family and three sporadic PCOS patients.The expression of LHCGRL638 P in granulosa-like tumor cell line(KGN)cells promoted cyclic adenosine monophosphate production and granulosa cell proliferation,indicating that LHCGRL638 P is an activating mutation.Lhcgr~(L642 P/L642 P)mice showed an irregular estrous cycle,reduced follicles with dynamic folliculogenesis,and increased testosterone(T),estradiol(E2),and dehydroepiandrosterone.Lhcgr~(+/L642 P)AIms1~(+/PB)mice displayed increased T and E2 but decreased late secondary and preovulatory follicles.We showed that activating mutation of LHCGR likely plays important roles in the pathophysiology of PCOS involving abnormal reproductive physiology,whereas ALMS1 deficiency may promote anovulatory infertility via elevated androgens,suggesting that the disturbed LHCGR and ALMS1 cooperatively induce PCOS phenotypes,characterized as anovulation and hyperandrogenemia frequently observed in PCOS patients with obesity. | Li Yu Lina Wang Wufan Tao Wenxiang Zhang Shuanghao Yang Jian Wang Jia Fei Rui Peng Yiming Wu Xiumei Zhen Hong Shao Weiyue Gu Rong Li Bai-Lin Wu Hongyan Wang | 2021 | Journal of Genetics and Genomics2021，48，5： | 3 |
| 5 | Atomically dispersed N-coordinated Fe-Fe dual-sites with enhanced enzyme-like activities显示文摘Replacement of enzymes with nanomaterials such as atomically dispersed metal catalysts is one of the most crucial steps in addressing the challenges in biocataiysis.Despite the breakthroughs of single-atom catalysts in enzyme-mimicking,a fundamental investigation on the development of an instructional strategy is still required for mimicking biatomic/multiatomic active sites in natural enzymes and constructing synergistically enhanced metal atom active sites.Herein,Fe_(2)NC catalysts with atomically dispersed Fe-Fe dual-sites supported by the metal-organic frameworks-derived nitrogen-doped carbon are employed as biomimetic catalysts to perform proof-of-concept investigation.The effect of Fe atom number toward typical oxidase(cytochrome C oxidase,NADH oxidase,and ascorbic acid oxidase)and peroxidase(NADH peroxidase and ascorbic acid peroxidase)activities is systematically evaluated by experimental and theoretical investigations.A peroxo-like O_(2) adsorption in Fe_(2)NC nanozymes could accelerate the O-O activation and thus achieve the enhanced enzyme-like activities.This work achieves the vivid simulation of the enzyme active sites and provides the theoretical basis for the design of high-performance nanozymes.As a concept application,a colorimetric biosensor for the detection of S^(2-) in tap water is established based on the inhibition of enzyme-like activity of Fe_(2)NC nanozymes. | Lei Jiao Wei Ye Yikun Kang Yu Zhang Weiqing Xu Yu Wu Wenling Gu Weiyu Song Yujie Xiong Chengzhou Zhu | 2022 | Nano Research2022，15，2： | 1 |
| 6 | Neurotoxicity of low-dose repeatedly intranasal instillation of nano- and submicron-sized ferric oxide particles in mice显示文摘 | Bing Wang Weiyue Feng Motao Zhu Yun Wang Meng Wang Yiqun Gu Hong Ouyang Huajian Wang Ming Li Yuliang Zhao Zhifang Chai Haifang Wang | 2009 | Journal of Nanoparticle Research2009，，1： | 1 |
| 7 | Cyclic RGD conjugated poly(ethylene glycol)- co -poly(lactic acid) micelle enhances paclitaxel anti-glioblastoma effect显示文摘 | Changyou Zhan Bing Gu Cao Xie Jin Li Yu Liu Weiyue Lu | 2009 | Journal of Controlled Release2009，，1： | 1 |
| 8 | Cyclic RGD conjugated poly(ethylene glycol)- co -poly(lactic acid) micelle enhances paclitaxel anti-glioblastoma effect显示文摘 | Changyou Zhan Bing Gu Cao Xie Jin Li Yu Liu Weiyue Lu | 2009 | Journal of Controlled Release2009，，1： | 1 |
| 9 | Cyclic RGD conjugated poly(ethylene glycol)- co -poly(lactic acid) micelle enhances paclitaxel anti-glioblastoma effect显示文摘 | Changyou Zhan Bing Gu Cao Xie Jin Li Yu Liu Weiyue Lu | 2009 | Journal of Controlled Release2009，，1： | 1 |
| 10 | Multi-parameters of Thermal Infrared Remote Sensing Anomalies of the Earthquake显示文摘Temporal and spatial anomalies associated with the Yushu earthquake, including the Outgoing Longwave Radiation( OLR), the Land Surface Temperature( LST) and surface temperature from the National Center for Environmental Prediction( NCEP) are studied using thermal infrared remote sensing data in this paper. All results confirmed the previous observations of thermal anomalies in the seismic region prior to this earthquake.Among the multi-parameter anomalies, the underground water temperature anomaly appeared first and lasted for the longest time; OLR anomaly,an infrared parameter which indicates the radiation characteristics of the land surface medium,was the first to be detected; LST anomalies appeared later than OLR. NCEP temperature indicates the average atmosphere temperature with a certain vertical thickness; therefore,it was the last detected anomaly. The anomalies of OLR and LST lasted for a similar time length and were all located in the south or southwest of the epicenter. | Lu Xian Meng Qingyan Gu Xingfa Zhang Xiaodong Xiong Pan Ma Weiyu Xie Tao | 2016 | Earthquake Research in China2016，30，4： | 1 |
| 11 | Cyclic RGD conjugated poly(ethylene glycol)- co -poly(lactic acid) micelle enhances paclitaxel anti-glioblastoma effect显示文摘 | Changyou Zhan Bing Gu Cao Xie Jin Li Yu Liu Weiyue Lu | 2009 | Journal of Controlled Release2009，，1： | 1 |
| 12 | Cyclic RGD conjugated poly(ethylene glycol)-co-poly(lactic acid)micelle enhances paclitaxel anti-glioblastoma effect显示文摘 | Zhan Changyou Gu Bing Xie Cao Li Jin Liu Yu Lu Weiyue | | 0，，： | 1 |
| 13 | Cyclic RGD conjugated poly(ethylene glycol)- co -poly(lactic acid) micelle enhances paclitaxel anti-glioblastoma effect显示文摘 | Changyou Zhan Bing Gu Cao Xie Jin Li Yu Liu Weiyue Lu | 2009 | Journal of Controlled Release2009，，1： | 1 |
| 14 | Acute toxicological impact of nano- and submicro-scaled zinc oxide powder on healthy adult mice显示文摘 | Bing Wang Weiyue Feng Meng Wang Tiancheng Wang Yiqun Gu Motao Zhu Hong Ouyang Junwen Shi Fang Zhang Yuliang Zhao Zhifang Chai Haifang Wang Jing Wang | 2008 | Journal of Nanoparticle Research2008，，2： | 1 |
| 15 | Cyclic RGD conjugated poly(ethylene glycol)- co -poly(lactic acid) micelle enhances paclitaxel anti-glioblastoma effect显示文摘 | Changyou Zhan Bing Gu Cao Xie Jin Li Yu Liu Weiyue Lu | 2009 | Journal of Controlled Release2009，，1： | 1 |
| 16 | Cyclic RGD conjugated poly(ethylene glycol)- co -poly(lactic acid) micelle enhances paclitaxel anti-glioblastoma effect显示文摘 | Changyou Zhan Bing Gu Cao Xie Jin Li Yu Liu Weiyue Lu | 2009 | Journal of Controlled Release2009，，1： | 1 |
| 17 | Development and evaluation of novel itraconazole-loaded intravenous nanoparticles显示文摘 | Wei Chen Bing Gu Hao Wang Jun Pan Weiyue Lu Huimin Hou | 2008 | International Journal of Pharmaceutics2008，，1： | 1 |
| 18 | Poly(ethylene glycol)-block-poly( d , l -lactide acid) micelles anchored with angiopep-2 for brain-targeting delivery显示文摘 | Jie Shen Changyou Zhan Cao Xie Qinggang Meng Bing Gu Chong Li Yingkai Zhang Weiyue Lu | 2011 | Journal of Drug Targeting2011，，3： | 1 |
| 19 | An in-situ NH_(4)^(+)-etched strategy for anchoring atomic Mo site on ZnIn_(2)S_(4) hierarchical nanotubes for superior hydrogen photocatalysis显示文摘Atomic sites co-catalyst (ASC) on photocatalytic materials possesses an attractive prospect to promote charge carrier separation and tune surface reaction kinetics,yet the synthesis of earth-abundant ASC under low temperature remains a great challenge.Here,a novel in-situ NH_(4)^(+)-etched strategy to anchor atomic Mo sites on ZnIn_(2)S_(4)hierarchical nanotubes (HNTs) with abundant mesopores under mild conditions for promoting charge carrier separation and enhancing light multi-reflections is developed for efficient photocatalytic H_(2) evolution.Density functional theory calculations and linear sweep voltammetry demonstrate that the well-defined Mo-S_(2)O_(1) sites with distinctive coordination configuration and electronic property contribute to the enhanced separation of photo-generated charge carriers and reduced Gibbs free energy for H_(2) evolution.Consequently,the well-defined MoSA-ZIS HNTs present an excellent photocatalytic activity with a rate of 29.9μmol h^(-1)(5.98 mmol g^(-1)h^(-1)),which is 7.3 times higher than that of ZnIn_(2)S_(4)nanosheets (NSs),to be among the best ZnIn_(2)S_(4)-based photocatalysts.The present strategy breaks the high-temperature limitation of conventional top-down thermal dissociation/emitting approach for anchoring non-noble metal atomic sites on catalyst support. | Yuguang Chao Weiyu Zhang Peng Zhou Hui Chen Shiyu Lu Menggang Li Qinghua Zhang Lin Gu Shaojun Guo | 2021 | Science China Chemistry2021，64，10： | 0 |
| 20 | Fine-Tuning Pyridinic Nitrogen in Nitrogen-Doped Porous Carbon Nanostructures for Boosted Peroxidase-Like Activity and Sensitive Biosensing显示文摘Carbon materials have been widely used as nanozymes in bioapplications,attributing to their intrinsic enzyme-like activities.Nitrogen(N)-doping has been explored as a promising way to improve the activity of carbon material-based nanozymes(CMNs).However,hindered by the intricate N dopants,the real active site of N-doped CMNs(N-CMNs)has been rarely investigated,which subsequently retards the further progress of high-performance N-CMNs.Here,a series of porous N-CMNs with well-controlled N dopants were synthesized,of which the intrinsic peroxidase(POD)like activity has a positive correlation with the pyridinic N content.Density functional theory calculations also reveal that pyridinic N boosts the intrinsic POD-like activity of N-CMNs.Pyridinic-N dopant can effectively promote the first H_(2)O desorption process in comparison with the graphitic and pyrrolic N,which is the key endothermic reaction during the catalytic process.Then,utilizing the optimized nanozymes with high pyridinic N content(NP-CMNs)and superior POD-like activity,a facile total antioxidant capacity(TAC)assay was developed,holding great promise in the quality assessment of medicine tablets and antioxidant food for healthcare and healthy diet. | Hongye Yan Linzhe Wang Yifeng Chen Lei Jiao Yu Wu Weiqing Xu Wenling Gu Weiyu Song Dan Du Chengzhou Zhu | 2020 | Research2020，，1： | 0 |
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