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PACS gene family-related neurological diseases: limited genotypes and diverse phenotypes

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World Journal of Pediatrics 2024年第1期20卷 82-91页

作者： Han Zhang Kai Gao Shuang Wang Yue-hua Zhang Zhi-Xian Yang Ye wu Yu-wu jiang Department of Pediatrics Peking University First Hospital.No.1 Xi'an Men StreetWest DistrictBeijing 100034China Bejing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases BeijingChina Children Epilepsy Center Peking University First HospitalBeijingChina Key Laboratory for Neuroscience Ministry of Education/National Health and Family Planning CommissionPeking UniversityBeijingChina Center of Epilepsy Beijing Institute for Brain DisordersBeijingChina

Background The PACS gene family has been demonstrated to be related to intracellular vesicular *** phenotypic manifestations caused by the pathogenic variants of PACS include epilepsy,intellectual disability/developmental delay,and malformations,such as facial *** We identified seven new cases with pathogenic or likely pathogenic PACS variants using next-generation *** information obtained from these patients was analyzed along with that obtained from previously reported *** With the inclusion of the newly diagnosed cases in this study,103 cases with PACS gene family-related neurological diseases were reported,of which 43 were PACS2-related cases and the remaining were PACSI-related *** patients had seizures,which have been reported to be effectively controlled by several types of anti-seizure medications(ASMs).The most efficacious and frequently prescribed ASMs included sodium valproate(43.3%,13/30),oxcarbazepine/carbamazepine(26.7%,8/30),and levetiracetam(20%,6/30).Almost all patients had intellectual disability/developmental *** most common pathogenic missense variants were PACSI ***203Trp and PACS2 *** addition,we report a patient carrying a likely pathogenic copy number variation(CNV)(de novo heterozygous deletion of chr14:105821380-106107443,286 kilobase,destroyed part of the furin-binding region domain and the protein structure after it)with more severe and refractory late-onset *** The clinical phenotypes of the different PACS heterozygous missense variants were *** pathogenic variant sites of PACSI and PACS2 were quite limited but located in different regions.A CNV destroying part of the PACS2 gene might also be *** findings may provide an important clue for further functional studies on the pathogenic mechanism of neurological disorders related to the PACS gene family.

关键词： Developmental delay Epilepsy Intellectual disability PACSI PACS2

A New Solution to Intrusion Detection Systems Based on Improved Federated-Learning Chain

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Computers, Materials & Continua 2024年第6期79卷 4491-4512页

作者： Chunhui Li hua jiang School of Computer and Electronic Information Guangxi UniversityNanning530000China Cyber Security and Information Center Guangxi UniversityNanning530000China

In the context of enterprise systems,intrusion detection(ID)emerges as a critical element driving the digital transformation of *** systems spanning various sectors of enterprises geographically dispersed,the necessity for seamless information exchange has surged *** existing cross-domain solutions are challenged by such issues as insufficient security,high communication overhead,and a lack of effective update mechanisms,rendering them less feasible for prolonged application on resource-limited *** study proposes a new cross-domain collaboration scheme based on federated chains to streamline the server-side *** this framework,individual nodes solely engage in training local data and subsequently amalgamate the final model employing a federated learning algorithm to uphold enterprise systems with efficiency and *** curtail the resource utilization of blockchains and deter malicious nodes,a node administration module predicated on the workload paradigm is introduced,enabling the release of surplus resources in response to variations in a node’s contribution *** encountering an intrusion,the system triggers an alert and logs the characteristics of the breach,facilitating a comprehensive global update across all nodes for collective *** results across multiple scenarios have verified the security and effectiveness of the proposed solution,with no loss of its recognition accuracy.

关键词： Cross-domain collaboration blockchain federated learning contribution value node management release slack resources

Finite-time composite learning control for nonlinear teleoperation systems under networked time-varying delays

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Science China(Information Sciences) 2024年第6期67卷 279-296页

作者： Yana YANG Huixin jiang Changchun hua Junpeng LI Institute of Electrical Engineering Yanshan University

The robust finite-time synchronization control problem is investigated for master-slave networked nonlinear telerobotics systems(NNTSs) in this article. Although there have been some research achievements on finite-time control for the NNTSs, these studies are based on the strong assumptions of communication time delays or can only achieve finite-time bounded convergence even when the external forces are *** and in view of the importance of these issues, a novel robust composite learning adaptive control scheme rendering the finite-time master-slave synchronization is proposed in this paper. In particular, the influence of time delays on finite-time convergence of the system is analyzed by employing the multi-dimension finite-time small-gain framework. Meanwhile, in order to achieve accurate and fast estimation of uncertain parameters of the system, both the online historical and the instantaneous data of the estimation data are explored to derive the new parameter adaptive law under a more realizable interval-excitation(IE) ***, the convergence of the position/force synchronization errors and the adaptive parameter estimation errors is obtained in finite time, and enhanced robustness of the closed-loop system will also be ***, the superior performance of the proposed control algorithms is validated by numerical simulations and hardware experiments.

关键词： networked teleoperation systems finite-time control exact parameter estimation small-gain theorem unknown communication delays

Nano polystyrene microplastics could accumulate in Nile tilapia(Oreochromis niloticus):Negatively impacts on the intestinal and liver health through water exposure

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Journal of Environmental Sciences 2024年第3期137卷 604-614页

作者： Di wu Xing Lu Li-Xue Dong Juan Tian Jin Deng Lei Wei hua Wen Shan Zhong Ming jiang School of Basic Medical Sciences Wuhan UniversityWuhan 430071China Key Laboratory of Freshwater Biodiversity Conservation Ministry of Agriculture and Rural AffairsYangtze River Fisheries Research InstituteChinese Academy of Fishery SciencesWuhan 430223China Hubei Province Key Laboratory of Allergy and Immunology Wuhan 430071China

Microplastics(MPs)have become a significant concern for their potential ***,the correlation between the size of plastic particles and their toxicity remains ***,we investigate the toxic effects of different sizes(80 nm,800 nm,8μm and 80μm)polystyrene MPs(PS-MPs)on the model organism Nile tilapia(Oreochromis niloticus).The results of bioluminescent imaging indicate that the 80 nm PS-MPs are more likely to invade the body.H&E staining shows severe damage on the intestinal villi and distinct hepatic steatosis in the 80 nm *** U labeling shows that the proliferation activity of intestinal and liver cells reduces significantly in the 80 nm *** gut microbiome analysis shows a severe imbalance of gut microbiota homeostasis in the 80 nm *** analysis of liver transcriptomics and metabolomics shows that the liver lipid metabolism is disordered in the 80 nm *** conclusion,this study confirms that the 80 nm PS-MPs are more likely to induce intestinal and liver *** the above lay the foundation for further study on the pathological damage of MPs to other organisms.

关键词： Microplastics(MPs) Intestinal toxicity Liver toxicity Gut microbiome Lipid metabolism

Establishing the link between D-mannose and juvenile grass carp(Ctenopharyngodon idella):Improved growth and intestinal structure associated with endoplasmic reticulum stress,mitophagy,and apical junctional complexes

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Animal Nutrition 2024年第3期18卷 450-463页

作者： Chong Zhang Lin Feng Pei wu Yang Liu Xiaowan Jin Hongmei Ren hua Li Fali wu Xiaoqiu Zhou Weidan jiang Animal Nutrition Institute Sichuan Agricultural UniversityChengdu 611130China Fish Nutrition and Safety Production University Key Laboratory of Sichuan ProvinceSichuan Agricultural UniversityChengdu 611130China Key Laboratory of Animal Disease-Resistance Nutrition Ministry of EducationMinistry of Agriculture and Rural AffairsKey Laboratory of Sichuan ProvinceChengdu 611130China

D-mannose,essential for protein glycosylation,has been reported to have immunomodulatory effects and to maintain intestinal flora *** addition to evaluating growth performance,we examined the impact of D-mannose on the structure of epithelial cells and apical junction complexes in the animal *** 1800 grass carp(16.20±0.01 g)were randomly divided into six treatments with six replicates of 50 fish each and fed with six different levels of D-mannose(0.52,1.75,3.02,4.28,5.50 and 6.78 g/kg diet)for 70 *** study revealed that D-mannose increased feed intake(P 0.05).D-mannose supplementation at 1.75 g/kg increased crude protein content in fish and lipid production value(P<0.05).D-mannose supplementation at 4.28 g/kg increased intestinal length,intestinal weight and fold height of grass carp compared to the control group(P<0.05).This improvement may be attributed to the phosphomannose isomerase(PMI)-mediated enhancement of *** study found that D-mannose supplementation at 4.28 or 3.02 g/kg reduced serum diamine oxidase activity or D-lactate content(P<0.05)and improved cellular and intercellular structures for the first *** improvement of cellular redox homeostasis involves alleviating endoplasmic reticulum(ER)stress through the inositol-requiring enzyme 1(IRE1),RNA-dependent protein kinase-like ER kinase(PERK),and activating transcription factor 6(ATF6)signaling *** alleviation of ER stress may be linked to the phosphomannomutase(PMM)-mediated enhancement of protein *** addition,ubiquitin-dependent[PTEN-induced putative kinase 1(PINK1)/Parkin]and ubiquitin-independent[BCL2-interacting protein 3-like(BNIP3L),BCL2-interacting protein 3(BNIP3),and FUN14 domain containing 1(FUNDC1)]mitophagy may play a role in maintaining cellular redox *** enhancement of intercellular structures includes enhancing tight junction and adherent junction structures,which may be closely associated with the small Rho GTPase pr

关键词： Grass carp(Ctenopharyngodon idella) D-mannose Intestine structure Endoplasmic reticulum stress Mitophagy Apical junctional complex

Effects of NO_(2) and SO_(2) on the secondary organic aerosol formation fromβ-pinene photooxidation

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Journal of Environmental Sciences 2024年第2期136卷 151-160页

作者： Xiangyu Zang Zhaoyan Zhang Yingqi Zhao Gang Li hua Xie Weiqing Zhang Guorong wu Xueming Yang Ling jiang Zhang Dayu School of Chemistry Dalian University of TechnologyDalian 116024China State Key Laboratory of Molecular Reaction Dynamics Dalian Institute of Chemical PhysicsChinese Academy of SciencesDalian 116023China University of Chinese Academy of Sciences Beijing 100049China Department of Chemistry School of ScienceSouthern University of Science and TechnologyShenzhen 518055China

Elucidating the effects of anthropogenic pollutants on the photooxidation of biogenic volatile organic compounds is crucial to understanding the fundamental mechanisms of secondary organic aerosol(SOA)***,the impacts of NO_(2)and SO_(2)on SOA formation from the photooxidation of a representative monoterpene,β-pinene,were investigated by a number of laboratory *** results indicated NO_(2)enhanced the SOA mass concentrations and particle number concentrations under both low and highβ-pinene *** could be rationalized that the increased O_(3)concentrations upon the NO_(x)photolysis was helpful for the generation of more amounts of O_(3)-oxidized products,which accelerated the SOA nucleation and *** with NO_(2),the promotion of the SOA yield by SO_(2)was mainly reflected in the increase of mass concentration,which might be due to the elimination of the newly formed particles by the initially formed *** observed low oxidation degree of SOA might be attributed to the fast growth of SOA,resulting in the uptake of less oxygenated gas-phase species onto the particle *** present findings have important implications for SOA formation affected by anthropogenic–biogenic interactions in the ambient atmosphere.

关键词： Secondary organic aerosol Volatile organic compound Anthropogenic pollutant Beta-pinene Photooxidation

Do nocturnal birds use acoustic and visual cues to avoid predation by bats?

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Integrative Zoology 2024年第3期19卷 524-537页

作者： Lixin GONG huan wu Zhiqiang WANG Hui wu jiang FENG Tinglei jiang Jilin Provincial Key Laboratory of Animal Resource Conservation and Utilization Northeast Normal UniversityChangchunChina Key Laboratory of Vegetation Ecology of Education Ministry Institute of Grassland ScienceNortheast Normal UniversityChangchunChina College of Life Science Jilin Agricultural UniversityChangchunChina

Anti-predation strategies are critical to animal survival and are fundamental to deciphering predator–prey *** an important defense strategy,sensory predator detection(such as through acoustic and visual cues)enables animals to assess predation risk and execute predator-avoidance behavior;however,there are limited studies on the anti-predation behavior of nocturnal *** prey of bats provides an excellent representative system for examining the anti-predation behavior of nocturnal ***,we broadcasted different types of echolocation calls of the bird-eating bat Ia io to two wild passerine birds,namely,Zosterops japonicus and Sinosuthora webbiana,that are preyed upon by ***,and presented the birds with individual bats under different light *** results showed that both bird species were able to perceive the low-frequency audible portion of the bats’echolocation calls;however,they did not exhibit escape responses to the acoustic *** the dark and under moonlit conditions,both bird species were unable to respond to active bats at close range and the birds only exhibited evasive flight behavior when bats approached or touched *** results suggest that nocturnal passerine birds may not be able to use acoustic or visual cues to detect bats and adopt evasive maneuvers to avoid *** work suggests that bat predation pressure may not elicit primary predator-avoidance responses in nocturnal passerine *** results provide new insights into the anti-predation behavior of nocturnal animals.

关键词： anti-predation behavior nocturnal animals sensory cues sensory defenses

Prevalence and clinical impact of sarcopenia in liver transplant recipients:A meta-analysis

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World Journal of Gastroenterology 2024年第8期30卷 956-968页

作者： Min-Jie jiang Mu-Chen wu Zhong-Hui Duan Jing wu Xiao-Tong Xu Juan Li Qing-hua Meng Department of Hepatology Beijing You-An HospitalCapital Medical UniversityBeijing 100069China Department of Emergency Beijing You-An HospitalCapital Medical UniversityBeijing 100069China

BACKGROUND The prevalence of sarcopenia in patients undergoing liver transplantation(LT)remains to be determined partly because of different diagnostic *** has recently been recognized as a new prognostic factor for predicting outcomes in LT *** To estimate the prevalence of sarcopenia and evaluate its clinical effect on LT *** This systematic search was conducted in PubMed,Web of Science,Embase,and Cochrane Library for original English-language articles that investigated the prevalence and influence of sarcopenia in patients undergoing LT from database inception to November 30,*** studies of the definition of sarcopenia that estimate sarcopenia prevalence and evaluate its effect on clinical outcomes and the risk of mortality were *** Twenty-five studies involving 7760 patients undergoing LT were *** pooled prevalence of sarcopenia in patients undergoing LT was 40.7%[95%confidence intervals(95%CI):32.1-49.6].The 1-,3-,and 5-year cumulative probab-ilities of post-LT survival in patients with preoperative sarcopenia were all lower than those without sarcopenia(P<0.05).Sarcopenia was associated with an increased risk of post-LT mortality in patients undergoing LT(adjusted hazard ratio:1.58;95%CI:1.21-2.07).Patients with preoperative sarcopenia had a longer intensive care unit stay,a high risk ratio of sepsis,and serious post-LT complications than those without *** Sarcopenia is prevalent in a substantial proportion of patients undergoing LT and is strongly and independently associated with higher a risk of mortality risk.

关键词： Sarcopenia Liver transplantation Mortality Clinical outcomes

Left ventricular thrombosis caused cerebral embolism during venoarterial extracorporeal membrane oxygenation support: A case report

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World Journal of Clinical Cases 2024年第5期12卷 973-979页

作者： Yao-Bang Bai Feng Zhao Zhen-hua wu Guo-Ning Shi Nan jiang Intensive Care Unit Department of Cardiac SurgeryTianjin Chest HospitalTianjin 300222China Department of Cardiac Surgery Tianjin Chest HospitalTianjin 300222China

BACKGROUND Venoarterial(VA)extracorporeal membrane oxygenation(ECMO),an effective short-term circulatory support method for refractory cardiogenic shock,is widely ***,retrospective analyses have shown that VA-ECMO-assisted cases were associated with a relatively high mortality rate of approximately 60%.Embolization in important organs caused by complications of left ventricular thrombosis(LVT)during VA-ECMO is also an important *** the incidence of LVT during VA-ECMO is not high,the consequences of embolization are *** SUMMARY A 37-year-old female patient was admitted to hospital because of fever for 4 d and palpitations for 3 *** excluding the diagnosis of coronary heart disease,we established a diagnosis of“clinically explosive myocarditis”.The patient still had unstable hemodynamics after drug treatment supported by VA-ECMO,with heparin for *** day 4 of ECMO support,a left ventricular thro-mbus attached to the papillary muscle root of the mitral valve was found by transthoracic *** ventricular decompression was performed and ECMO was successfully removed,but the patient eventually died of multiple cerebral *** LVT with high mobility during VA-ECMO may cause embolism in important ***,a"wait and see"strategy should be avoided.

关键词： Venoarterial extracorporeal membrane oxygenation Left ventricular thrombosis Cerebral embolism Magnetic Resonance Imaging Therapy Case report