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ccdc141 is required for left-right axis development by regulating cilia formation in the Kupffer's vesicle of zebrafish

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Journal of Genetics and Genomics 2024年第9期51卷 934-946页

作者： Pengcheng Wang Wenxiang Shi Sijie Liu Yunjing Shi Xuechao Jiang Fen Li sun Chen kun sun Rang Xu Department of Pediatric Cardiology Xinhua HospitalShanghai Jiao Tong University School of MedicineShanghai 200092China Department of Cardiovascular Medicine Heart Failure CenterRuijin HospitalRuijin Hospital Lu Wan BranchShanghai Jiao Tong University School of MedicineShanghai 200025China Scientific Research Center Xinhua HospitalShanghai Jiao Tong University School of MedicineShanghai 200092China Department of Pediatric Cardiology Shanghai Children's Medical CenterShanghai Jiao Tong University School of MedicineShanghai 200127China

Laterality is a crucial physiological process intricately linked to the cilium-centrosome complex during embryo *** in the process can result in severe organ ***-coil domain containing 141(CCDC141)has been previously known as a centrosome-related gene,but its role in left-right(LR)asymmetry has not been *** this study,we utilize the zebrafish model and human exome analysis to elucidate the function of ccdc141 in laterality *** knockdown of ccdc141 in zebrafish disrupts early LR signaling pathways,cilia function,and Kupffer's vesicle *** ccdc141-knockdown embryos exhibiting aberrant LR patterns,ccdc141-null mutants show no apparent abnormality,suggesting a genetic compensation response *** parallel,we observe a marked reduction inα-tubulin acetylation levels in the ccdc141 *** treatment with histone deacetylase(HDAC)inhibitors,particularly the HDAC6 inhibitor,rescues the ccdc141 crispant ***,exome analysis of 70 patients with laterality defects reveals an increased burden of CCDC141 mutations,with in-vivo studies verifying the pathogenicity of the patient mutation *** findings highlight the critical role of ccdc141 in ciliogenesis and demonstrate that CCDC141 mutations lead to abnormal LR patterns,identifying it as a candidate gene for laterality defects.

关键词： Left-right axis Cilia WES Laterality defects Zebrafish CCDC141

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Protein C deficiency with venous and arterial thromboembolic events

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World Journal of Clinical Cases 2024年第12期12卷 2000-2003页

作者： Nan Zhang Dong-kun sun Xu Tian Xin-Yu Zheng Tong Liu Department of Cardiology Tianjin Key Laboratory of Ionic-Molecular Function of Cardiovascular DiseaseTianjin Institute of CardiologyThe Second Hospital of Tianjin Medical UniversityTianjin 300211China

Protein C(PC)is a key component of the vitamin K-dependent coagulation *** exerts anticoagulant effects by inactivating factors V and *** or inherited PC deficiency results in a prothrombotic state,with presentations varying from asymptomatic to venous ***,there has been an increasing number of reports linking PC deficiency to arterial thromboembolic events,such as myocardial infarction and ischemic *** editorial focuses on the association between PC deficiency and thromboembolism,which may provide some insights for treatment strategy and scientific research.

关键词： Protein C deficiency Venous thromboembolism Myocardial infarction Editorial Arterial thromboembolism

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Decadal shifts in Qingzang Plateau lake carbon dynamics (1970-2020): From predominant carbon sources to emerging sinks

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Environmental Science and Ecotechnology 2024年第5期21卷 123-132页

作者： Di Shen Yu Li Yafeng Wang Shouliang Huo Yong Liu Junjie Jia Shuoyue Wang kun sun Yang Gao College of Earth and Environment Science Lanzhou UniversityLanzhou730000PR China State Key Laboratory of Tibetan Plateau Earth System Environment and Resources(TPESER)Institute of Tibetan Plateau ResearchChinese Academy of SciencesBeijing100101PR China Chinese Research Academy of Environmental Sciences Beijing100012PR China College of Environmental Sciences and Engineering Peking UniversityBeijing1008714PR China Key Laboratory of Ecosystem Network Observation and Modeling Institute of Geographic Sciences and Natural Resources ResearchChinese Academy of SciencesBeijing100101PR China College of Resources and Environment University of Chinese Academy of SciencesBeijing100049PR China

The evasion of carbon dioxide(CO_(2))from lakes significantly influences the global carbon *** global climatic transformations,the role of Qingzang Plateau(QZP)lakes as carbon(C)sources or sinks remains a subject of ***,accurately quantifying their contribution to the global carbon budget presents a formidable ***,spanning half a century(1970e2020),we utilize a synthesis of literature and empirical field data to assess the CO_(2) exchange flux of QZP *** find markedly higher CO_(2) exchange flux in the southeast lakes than that in the northern and western regions from 1970 to *** this time,both freshwater and saltwater lakes served primarily as carbon *** annual CO_(2) exchange flux was estimated at 2.04±0.37 Tg(Tg)C yr1,mainly influenced by temperature *** CO_(2) exchange flux patterns underwent a geographical inversion between 2000 and 2020,with increased levels in the west and decreased levels in the ***,CO_(2) emissions from freshwater lakes diminished,and certain saltwater lakes in the QTP transitioned from carbon sources to *** 2000 to 2020,the annual CO_(2) exchange flux from QZP lakes is estimated at 1.34±0.50 Tg C yr1,with solar radiation playing a more pronounced role in carbon ***,over the past five decades,QZP lakes have generally functioned as carbon ***,the total annual CO_(2) emissions have declined since the year 2000,indicating a potential shift trend from being a carbon source to a sink,mirroring broader patterns of global climate *** findings not only augment our understanding of the carbon cycle in plateau aquatic systems but also provide crucial data for refining China's carbon budget.

关键词： Carbon source Carbon sink Carbon exchange flux Qingzang Plateau Global climate change

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Prognosis and outcome of intrauterine treatment of fetuses with critical congenital heart disease

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Chinese Medical Journal 2024年第12期137卷 1431-1436页

作者： Liqing Zhao Lei Wang Hongping Xia Yurong Wu Xianting Jiao Hong Zhu sun Chen kun sun Department of Pediatric Cardiology Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China Department of Diagnosis and Treatment Center for in utero Pediatric Disease Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China Department of Neonatology Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China

Background:Intrauterine valvuloplasty is an innovative therapy,which promotes ventricular growth and function in some congenital heart diseases(CHDs).The technique remains challenging and can only be performed in a few *** study aimed to assess the feasibility and mid-term outcomes of fetal cardiac intervention(FCI)in fetuses with critical CHD in an experienced tertiary ***:Five fetal aortic valvuloplasty(FAV)or fetal pulmonary valvuloplasty(FPV)procedures were performed in our fetal heart center between August 2018 and May *** success was defined as crossing the aortic or pulmonary valve and balloon inflation,followed by evidence of increased blood flow across the valve and/or new ***-up clinical records and echocardiography were obtained during the prenatal and postnatal ***:Five fetuses received FAV or FPV,including critical aortic stenosis(n=2)and pulmonary atresia with intact ventricular septum(n=3).The mean maternal age was 33.0±2.6 *** median gestational age(GA)at diagnosis was 24 weeks(range,22-26 weeks).The median GA at intervention was 29 weeks(range,28-32 weeks).All five cases underwent successful or partially successful *** patient had pulmonary valve perforation without balloon *** procedure-related deaths or significant complications ***,one neonatal death occurred due to heart and renal *** median follow-up period was 29.5 months(range,8.0-48.0 months).The four surviving patients had achieved biventricular circulation,exhibited improved valve,and ventricular development at the last follow-up ***:Intrauterine FCI could be performed safely with good prognosis in critical CHD.

关键词： Fetal cardiac intervention Valvuloplasty Pulmonary atresia Critical aortic stenosis Congenital heart disease

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基于图拉普拉斯正则化的PET图像核重建方法

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电子学报 2024年第1期52卷 118-128页

作者：盛玉霞孙坤柴利武汉科技大学信息科学与工程学院湖北武汉430081 浙江大学控制科学与工程学院浙江杭州310027

正电子发射断层成像(Positron Emission Tomography,PET)在很多疾病的早期诊断中有重要的作用,PET图像重建的难点之一是如何在保持重建图像中病灶边缘特性的同时具有良好的去噪性能.针对此问题,本文提出了一种结合图拉普拉斯正则化和深... 详细信息

正电子发射断层成像(Positron Emission Tomography,PET)在很多疾病的早期诊断中有重要的作用,PET图像重建的难点之一是如何在保持重建图像中病灶边缘特性的同时具有良好的去噪性能.针对此问题,本文提出了一种结合图拉普拉斯正则化和深度图像先验的PET图像核重建方法 .设计了改进的U-net神经网络,将PET前向投影模型中的核系数表示为神经网络的输出;通过先验图像构建图拉普拉斯矩阵,重建问题被建模为基于神经网络的带图拉普拉斯正则化项的最大似然函数优化问题.利用优化转移方法导出了收敛的迭代重建算法,每一次迭代包括由核重建方法更新图像和利用神经网络更新核系数两个步骤.仿真和临床实验结果表明,本文提出的方法在不同的指标下都有更好的重建效果,优于已有核重建方法以及最新的基于深度系数先验的重建方法 .

关键词： PET 图像重建核方法深度图像先验图拉普拉斯正则化

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Climate change reductions in lake ice cover duration and thickness help regulate the carbon sink potential of plateau type lakes

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National Science Open 2024年第5期3卷 114-129页

作者： Di Shen Yafeng Wang Junjie Jia Shuoyue Wang kun sun Yang Gao College of Earth and Environment Science Lanzhou UniversityLanzhou 730000China State Key Laboratory of Tibetan Plateau Earth System Environment and Resources(TPESER)Institute of Tibetan Plateau ResearchChinese Academy of SciencesBeijing 100101China Key Laboratory of Ecosystem Network Observation and Modeling Institute of Geographic Sciences and Natural Resources ResearchChinese Academy of SciencesBeijing 100101China College of Resources and Environment University of Chinese Academy of SciencesBeijing 100049China

Lake ice changes in winter under the influence of global climate change,but how lake ice changes will regulate water gross primary productivity(GPP)and carbon sequestration capacity is still ***,we evaluated and analyzed the geographic spatial pattern and dynamic changes of lake ice and GPP on the Qinghai-Tibetan Plateau(QTP)in the past 20 *** show that lake ice duration on the QTP is 123.36±2.43 d on average,although longer for lakes at higher altitudes,of moderate size,and with shallower *** ice thickness is between 55-66 cm on average,and its GPP on the QTP is between 0.17-3.35 g C m^(-2)d^(-1).In the context of global climate change,reductions in lake ice cover duration and changes in ice thickness on the QTP increased phytoplankton GPP during the winter freeze period while decreasing Carbon dioxide(CO_(2))emissions during the melting period.

关键词： Qinghai-Tibetan Plateau lake ice gross primary productivity carbon sequestration

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高超声速火箭橇凿削磨损机理分析

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西安交通大学学报 2024年第8期58卷 136-144页

作者：陈科儒孙琨陈诚齐博毅党峰西安交通大学金属材料强度国家重点实验室西安710049 中国兵器工业试验测试研究院西华阴714200陕西安交通大学航天航空学院西安710049

针对高超声速下火箭橇运行过程中凿削磨损产生的机理模糊不清问题,基于有限元-光滑粒子流体动力学法(FEM-SPH法),利用有限元软件ABAQUS构建了三维火箭橇靴-轨模型,FEM-SPH法是一种既能保证计算精度,又能克服网格畸变的新型模拟方法。在... 详细信息

针对高超声速下火箭橇运行过程中凿削磨损产生的机理模糊不清问题,基于有限元-光滑粒子流体动力学法(FEM-SPH法),利用有限元软件ABAQUS构建了三维火箭橇靴-轨模型,FEM-SPH法是一种既能保证计算精度,又能克服网格畸变的新型模拟方法。在该模型中,滑靴和轨道所使用的材料分别为VascoMax 300钢、AISI 1080钢,并赋予各自材料属性,通过设定航向速度为2000 m/s,竖直速度为2.5 m/s以及温度为673 K,模拟了高超声速下靴-轨凿削磨损现象的演变过程。模拟结果表明:凿削磨损是在高温高应力作用下和靴-轨之间形成嵌入式结构后,经过持续地破坏滑靴底部所产生的,最终形成泪滴状蚀坑。进一步分析表明,竖直速度和温度的增加都会提高凿削发生的概率。在航向速度为2000 m/s、初始温度为673 K时,竖直速度超过1.50 m/s就会发生凿削现象;在航向速度为2000 m/s、竖直速度为2.5 m/s,温度超过293 K就会发生凿削现象。该研究成果不仅有助于高超声速下靴-轨凿削磨损的产生机理进一步完善,还为高超声速火箭橇试验的安全发射提供了新的理论支持。

关键词：火箭橇试验凿削现象有限元-光滑粒子流体动力学法竖直速度温度

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基于LBM水力裂缝中支撑剂颗粒团簇升阻力系数研究

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工程力学 2024年第3期41卷 241-249页

作者：张涛吴春燕孙堃卢聪李聪西南石油大学石油与天然气工程学院成都610000 空气动力学国家重点实验室绵阳621000

水力压裂中,裂缝壁面对支撑剂团在狭窄裂缝中输送沉降时的受力有重要影响。该文基于LBM建立颗粒流动数值模型,构建星型、三棱柱型、长方体型颗粒团簇模型,研究颗粒团簇在不同雷诺数Re、颗粒团簇与壁面相对距离H/d下的升阻力变化规律。... 详细信息

水力压裂中,裂缝壁面对支撑剂团在狭窄裂缝中输送沉降时的受力有重要影响。该文基于LBM建立颗粒流动数值模型,构建星型、三棱柱型、长方体型颗粒团簇模型,研究颗粒团簇在不同雷诺数Re、颗粒团簇与壁面相对距离H/d下的升阻力变化规律。结果表明:壁面影响下,随Re的增大,颗粒团簇的曳力系数、升力系数均减小,且壁面方向升力系数总小于无壁面方向的结果;单颗粒曳力系数与团簇平均曳力系数的比值随Re的增加而减小,随壁面边界效应的影响而增大;不同位置的颗粒曳力系数比值表现为前排颗粒减小、中间颗粒先增大后减小、近壁面颗粒逐渐增大。随H/d的增大,颗粒团簇远离壁面,边界影响减弱,曳力系数减小程度逐渐减弱;前排及近壁面颗粒曳力系数比值与升力系数随之减小;远壁面颗粒逐渐靠近壁面,曳力系数比值增大。壁面边界效应会加剧近壁面颗粒受力,且外侧颗粒对内部颗粒受力有屏蔽作用。

关键词： LBM 颗粒团簇曳力系数升力系数壁面效应水力裂缝

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间伐后混交不同比例亮叶桦和毛竹对杉木生长的影响

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林草资源研究 2024年第2期 124-132页

作者：孙昆吴大瑜孙洪刚中国林业科学研究院亚热带林业研究所杭州311400 浙江省庆元县自然资源和规划局浙江庆元323800

科学合理的混交能够恢复土壤地力,提高林分生产力,改善低质低效林,实现对杉木人工林的科学高效经营。通过对杉木人工林进行抚育间伐,以不同混交比例营造杉木-亮叶桦(1杉1桦、2杉1桦、3杉1桦)和杉木-毛竹(1杉1毛、2杉1毛、3杉1毛)混交林... 详细信息

科学合理的混交能够恢复土壤地力,提高林分生产力,改善低质低效林,实现对杉木人工林的科学高效经营。通过对杉木人工林进行抚育间伐,以不同混交比例营造杉木-亮叶桦(1杉1桦、2杉1桦、3杉1桦)和杉木-毛竹(1杉1毛、2杉1毛、3杉1毛)混交林,探究混交对杉木个体生长特征和功能性状的影响。结果表明:1)与纯林相比,混交增加了林木竞争强度;杉木与亮叶桦混交时的林木竞争强度小于与毛竹混交。2)杉木与亮叶桦、毛竹混交均提高了杉木的叶面积指数。3)随杉木保留密度的增加,土壤中速效氮、有效磷和有机质含量随之下降;相比纯林,混交使土壤速效氮含量下降,但提高了土壤有效磷含量。4)杉木单株材积随杉木保留密度的增加而下降,林分蓄积反之。杉木-亮叶桦、杉木-毛竹混交林中,杉木单株材积和林分蓄积均高于自身纯林;杉木-亮叶桦混交林中杉木单株材积和林分蓄积同样大于杉木-毛竹混交林。5)Hegyi竞争指数与杉木单株材积呈极显著负相关(P<0.01);杉木单株材积与叶面积呈显著正相关(P<0.05);有效磷与单株材积和林分蓄积呈极显著正相关(P<0.01),土壤有机质含量与林分蓄积呈显著正相关(P<0.05)。综上,杉木-亮叶桦混交林在各方面要优于杉木-毛竹混交林;且杉木与亮叶桦混交比为1∶1时,对杉木单株生长促进效果最佳,有利于杉木大径材培育,促进杉木林分质量的提高;同时应兼顾林分产量,杉木与亮叶桦混交比为3∶1时,其杉木林分蓄积实现最大化。

关键词：杉木 Hegyi竞争指数叶面积指数有效磷林分蓄积

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Chinese pangolins (Manis pentadactyla) are not functionally extinct in China

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Zoological Research(Diversity and Conservation) 2024年第1期1卷 79-81页

作者： Zhi-Cheng Liu Peng Cen Jian-kun sun Fu-Hua Zhang Amna Mahmood Chen Lei Shi-Bao Wu School of Life Sciences South China Normal UniversityGuangzhouGuangdong 510631China

DEAR EDITOR,The key features of functional extinction include continuous reproductive failure,lack of new recruitment,and persistent population decline(Jarićet al.,2016;Roberts et al.,2017).However,the conclusion of functional extinction for the Chinese pangolin(Manis pentadactyla)by the China Biodiversity Conservation and Green Development Foundation(CBC-GDF)based on preliminary field information is unconvincing as they did not assess reproductive function or new individual recruitment of Chinese pangolin populations(Supplementary Materials).

关键词： extinction Roberts Manis

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