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RISS 인기검색어
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- 저자 : Yongjia Chen (검색결과 3 건)
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Anhai Chen,Chufeng He,Yong Feng,Jie Ling,Xin Peng,Xianlin Liu,Shuang Mao,Yongjia Chen,Mengyao Qin,Shuai Zhang,Yijiang Bai,Jian Song,Zhili Feng,Lu Ma,Dinghua He,Lingyun Mei1 대한이비인후과학회 2023 Clinical and Experimental Otorhinolaryngology Vol.16 No.4
Objectives. Branchio-oto syndrome (BOS) primarily manifests as hearing loss, preauricular pits, and branchial defects. EYA1is the most common pathogenic gene, and splicing mutations account for a substantial proportion of cases. However,few studies have addressed the structural changes in the protein caused by splicing mutations and potential pathogenicfactors, and several studies have shown that middle-ear surgery has limited effectiveness in improving hearing in thesepatients. BOS has also been relatively infrequently reported in the Chinese population. This study explored the ge-netic etiology in the family of a proband with BOS and provided clinical treatment to improve the patient’s hearing. Methods. We collected detailed clinical features and peripheral blood samples from the patients and unaffected individualswithin the family. Pathogenic mutations were identified by whole-exome sequencing and cosegregation analysis andclassified according to the American College of Medical Genetics and Genomics guidelines. Alternative splicing wasverified through a minigene assay. The predicted three-dimensional protein structure and biochemical experimentswere used to investigate the pathogenicity of the mutation. The proband underwent middle-ear surgery and was fol-lowed up at 1 month and 6 months postoperatively to monitor auditory improvement. Results. A novel heterozygous EYA1 splicing variant (c.1050+4 A >C) was identified and classified as pathogenic (PVS1(RNA),PM2, PP1). Skipping of exon 11 of the EYA1 pre-mRNA was confirmed using a minigene assay. This mutation mayimpair EYA1-SIX1 interactions, as shown by an immunoprecipitation assay. The EYA1-Mut protein exhibited cellularmislocalization and decreased protein expression in cytological experiments. Middle-ear surgery significantly improvedhearing loss caused by bone-conduction abnormalities in the proband. Conclusion. We reported a novel splicing variant of EYA1 in a Chinese family with BOS and revealed the potential molec-ular pathogenic mechanism. The significant hearing improvement observed in the proband after middle-ear surgeryprovides a reference for auditory rehabilitation in similar patients.
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Enhanced dielectric and nonohmic properties of SrTiO3-modified CaCu3Ti4O12 ceramics
Zhao Jiangpeng,Sun Li,Cao Ensi,Hao Wentao,Zhang Yongjia,Chen Jianbin,Ju Lin 한국물리학회 2022 Current Applied Physics Vol.36 No.-
Dielectric and nonohmic properties of CaCu3Ti4O12 (CCTO) ceramics can be modified by addition of SrTiO3 (STO) in different molar proportions which were fabricated by a modified sol-gel method. XRD results indicated that all modified ceramics showed mixed phase consisting of both CCTO and STO. SEM images and grain size distribution probability also presented the change of microstructure with the addition of STO. The dielectric loss of the CCTO/0.4STO ceramics sintered at 1000 ◦C can be lower than 0.02 in a wide frequency (1 kHz–10 kHz), especially at 1 kHz, the dielectric loss of this sample is as low as 0.012. Furthermore, excellent nonlinear I–V electrical characteristic (high breakdown voltage to 54.15 kV/cm for CCTO/0.4STO sintered at 1000 ◦C) was observed as well. All the results indicated that the addition of STO does improve the dielectric properties and nonohmic characteristics of CCTO ceramics dramatically.
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Modeling of ammonia-based wet flue gas desulfurization in the spray scrubber
Yong Jia,Qin Zhong,Xuyou Fan,Qianqiao Chen,Haibo Sun 한국화학공학회 2011 Korean Journal of Chemical Engineering Vol.28 No.4
A mathematical model of ammonia-based wet flue gas desulfurization process was developed based on the double film theory. The calculated results of the desulfurization system for two 220 t·h^−1 boilers per unit by this model were compared to that of corresponding measured data. It was found that the calculated results agree well with the measured data for the operating conditions of pH, liquid/gas ratio and SO_2 concentration. This model can provide predictions of the absorption performance of an ammonia-based wet flue gas desulfurization process and appears to be helpful for designing scrubbers for SO_2 absorption with ammonia absorbent.
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