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- 저자 : Bu Shanshan (검색결과 4 건)
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A brain somatic RHEB doublet mutation causes focal cortical dysplasia type II
Shanshan Zhao,Zhenghui Li,Muxian Zhang,Lingliang Zhang,Honghua Zheng,Jinhuan Ning,Yanyan Wang,Feng-Peng Wang,Xiaobin Zhang,Hexia Gan,Yuanqing Wang,Xian Zhang,Hong Luo,Guojun Bu,Huaxi Xu,Yi Yao,Yun-wu 생화학분자생물학회 2019 Experimental and molecular medicine Vol.51 No.-
Focal cortical dysplasia type II (FCDII) is a cerebral cortex malformation characterized by local cortical structure disorganization, neuronal dysmorphology, and refractory epilepsy. Brain somatic mutations in several genes involved in the PI3K/AKT/mTOR pathway are associated with FCDII, but they are only found in a proportion of patients with FCDII. The genetic causes underlying the development FCDII in other patients remain unclear. Here, we carried out whole exome sequencing and targeted sequencing in paired brain–blood DNA from patients with FCDII and identified a brain somatic doublet mutation c.(A104T, C105A) in the Ras homolog, mTORC1 binding (RHEB) gene, which led to the RHEB p.Y35L mutation in one patient with FCDII. This RHEB mutation carrier had a dramatic increase of ribosomal protein S6 phosphorylation, indicating mTOR activation in the region of the brain lesion. The RHEB p.Y35L mutant protein had increased GTPλS-binding activity compared with wild-type RHEB. Overexpression of the RHEB p. Y35L variant in cultured cells also resulted in elevated S6 phosphorylation compared to wild-type RHEB. Importantly, in utero electroporation of the RHEB p.Y35L variant in mice induced S6 phosphorylation, cytomegalic neurons, dysregulated neuron migration, abnormal electroencephalogram, and seizures, all of which are found in patients with FCDII. Rapamycin treatment rescued abnormal electroencephalograms and alleviated seizures in these mice. These results demonstrate that brain somatic mutations in RHEB are also responsible for the pathogenesis of FCDII, indicating that aberrant activation of mTOR signaling is a primary driver and potential drug target for FCDII.
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Wang Mouhao,Bu Shanshan,Zhou Bing,Li Zhenzhong,Chen Deqi 한국원자력학회 2023 Nuclear Engineering and Technology Vol.55 No.3
Fully Ceramic Microencapsulated (FCM) fuel is emerging advanced fuel material for the future nuclear reactors. The fuel pellet in the FCM fuel is composed of matrix and a large number of TRistructural- ISOtopic (TRISO) fuel particles which are randomly dispersed in the SiC matrix. The minimum layer thickness in a TRISO fuel particle is on the order of 105 m, and the length of the FCM pellet is on the order of 102 m. Hence, the heat transfer in the FCM pellet is a multi-scale phenomenon. In this study, three multi-scale heat conduction models including the Multi-region Layered (ML) model, Multi-region Non-layered (MN) model and Homogeneous model for FCM pellet were constructed. In the ML model, the random distributed TRISO fuel particles and coating layers are completely built. While the TRISO fuel particles with coating layers are homogenized in the MN model and the whole fuel pellet is taken as the homogenous material in the Homogeneous model. Taking the results by the ML model as the benchmark, the abilities of the MN model and Homogenous model to predict the maximum and average temperature were discussed. It was found that the MN model and the Homogenous model greatly underestimate the temperature of TRISO fuel particles. The reason is mainly that the conventional equivalent thermal conductivity (ETC) models do not take the internal heat source into account and are not suitable for the TRISO fuel particle. Then the improved ETCs considering internal heat source were derived. With the improved ETCs, the MN model is able to capture the peak temperature as well as the average temperature at a wide range of the linear powers (165 W/ cm~ 415 W/cm) and the packing fractions (20%e50%). With the improved ETCs, the Homogenous model is better to predict the average temperature at different linear powers and packing fractions, and able to predict the peak temperature at high packing fractions (45%e50%).
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Lian Qiang,Tang Simiao,Zhu Longxiang,Zhang Luteng,Sun Wan,Bu Shanshan,Pan Liangming,Tian Wenxi,Qiu Suizheng,Su G.H.,Wu Xinghua,Wang Xiaoyu 한국원자력학회 2023 Nuclear Engineering and Technology Vol.55 No.11
Blanket is of vital importance for engineering application of the fusion reactor. Nuclear heat deposition in materials is the main heat source in blanket structure. In this paper, the three-dimensional method for thermalhydraulics/ neutronics coupling analysis is developed and applied for the full-scale module of the helium-cooled ceramic breeder tritium breeding blanket (HCCB TBB) designed for China Fusion Engineering Test Reactor (CFETR). The explicit coupling scheme is used to support data transfer for coupling analysis based on cell-to-cell mapping method. The coupling algorithm is realized by the user-defined function compiled in Fluent. The threedimensional model is established, and then the coupling analysis is performed using the paralleled Coupling Analysis of Thermal-hydraulics and Neutronics Interface Code (CATNIC). The results reveal the relatively small influence of the coupling analysis compared to the traditional method using the radial fitting function of internal heat source. However, the coupling analysis method is quite important considering the nonuniform distribution of the neutron wall loading (NWL) along the poloidal direction. Finally, the structure optimization of the blanket is carried out using the coupling method to satisfy the thermal requirement of all materials. The nonlinear effect between thermal-hydraulics and neutronics is found during the blanket structure optimization, and the tritium production performance is slightly reduced after optimization. Such an adverse effect should be thoroughly evaluated in the future work.
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Numerical analysis on two-phase flow-induced vibrations at different flow regimes in a spiral tube
Yang Guangchao,Yu Xiaofei,Zhang Yixiong,Chen Guo,Bu Shanshan,Zhang Ke,Chen Deqi 한국원자력학회 2024 Nuclear Engineering and Technology Vol.56 No.5
Spiral tubes are used in a wide range of applications and it is significant to understand the vibration introduced by two-phase flow in spiral tubes. In this paper, the numerical method is used to study the vibration induced by the gas-liquid two-phase flow in a spiral tube with different flow regimes. The pressure fluctuation characteristics at the pipe wall and the solid vibration response characteristics are obtained. The results show that the motion of small bubbles in bubbly flow leads to small pressure fluctuations with low-frequency broadband (0–50 Hz). The motion of the gas plug in the plug flow causes small amplitude periodic pressure fluctuation with a shortened low-frequency broadband (0–15 Hz) compared to the bubbly flow. The motion of the gas slug in the slug flow causes large periodic fluctuations in pressure with a significant dominant frequency (6–7 Hz). The wavy flow is very stable and has a distinct main frequency (1–2 Hz). The vibration regime in the bubbly flow and wave flow are close to the first-order mode, and the vertical vibrating component is dominant. The plug flow and slug flow excite higher-order vibration modes, and the lateral vibration component plays more important part in the vibration response.
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