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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.
Xianlin Li,Biao Huang,Tairan Chen,Ying Liu,Sicong Qiu,Jing Zhao 대한기계학회 2017 JOURNAL OF MECHANICAL SCIENCE AND TECHNOLOGY Vol.31 No.1
We investigated the influence of geometrical parameters of the orifice plate on the cavitation structures, and optimized these parameters by using a surrogate-based model with special emphasis on the concentration of hydroxyl radical released. The results show that for the orifice plate of the hydrodynamic cavitation system, the possible location of the inception of the cavity spreads to throat and divergent section of the venturi geometry. Based on the surrogate model and global sensitivity assessment, the diameter of throat D t and diameter of inlet D in significantly influenced the size of the cavity, while the length of throat L t had little effect on both cavitation intensity and flow rate. It should be noted that when L t is decreased, the size of cavity would be slightly decreased but the flow rate increased clearly. The increase of the diverging section is in favor of the size of cavity. By comparing the experimental measurements on the concentration of Methylene blue, the optimum geometry of the orifice plate for best cavitational activity is proposed.
Libing Chen,Ruihong Liang,Genshui Wang,Hengchang Nie,Zhiyong Zhou,Fei Cao,Xianlin Dong 한국물리학회 2014 Current Applied Physics Vol.14 No.11
Pure and Nb-doped Pb(Zr1xTix)O3 (x ¼ 0.47, 0.48, 0.50) ceramics were prepared by conventional solidstate reaction technique. Dielectric anomalies are observed in both kinds of samples near room temperature. The anomalies could be depressed by donor doping and prefer to be significant in ceramics with tetragonal crystallographic phase. Phase transition mechanism and domain wall pinning effect are proposed to explain this anomaly, and the former is considered as the dominated reason. Further results of the pyroelectric measurements confirm the existence of the ferroelectriceferroelectric phase transition.
Depoling of porous Pb0.99(Zr0.95Ti0.05)0.98Nb0.02O3 ferroelectric ceramics under shock wave load
Ningbo Feng,Hengchang Nie,Xuefeng Chen,Genshui Wang,Xianlin Dong,Hongliang He 한국물리학회 2010 Current Applied Physics Vol.10 No.6
Poled Pb0.99(Zr0.95Ti0.05)0.98Nb0.02O3 ferroelectric ceramics in different porosity depoled under shock wave load. Porosity and material dielectric characteristic effects on the depoling current evolution were discussed. The results showed that under open circuit condition, the dielectric constant of all samples exhibited obvious increase, but as the porosity increased, the dielectric constant decreased. As a result of lower dielectric constant of porous samples during depoling process, the charge-releasing rate is faster than that of dense sample.
Mechanical induced electrical failure of shock compressed PZT95/5 ferroelectric ceramics
Hengchang Nie,Jia Yang,Xuefeng Chen,Fuping Zhang,Yin Yu,Genshui Wang,Yusheng Liu,Hongliang He,Xianlin Dong 한국물리학회 2017 Current Applied Physics Vol.17 No.4
A comparative experimental study of shock compressed Pb0.99(Zr0.95Ti0.05)0.98Nb0.02O3 (hereafter referred to as PZT95/5) ferroelectric ceramics within different insulation materials were performed in this study. It was found that the insulating materials play a significant role in the electrical failure behavior of PZT95/5 ferroelectric ceramic. The electrical breakdown behavior of PZT95/5 ferroelectric ceramics within epoxy resin was observed to aggravate at low temperature 55 C. Porous PZT95/5 ferroelectric ceramic was found to exhibit lower failure probability than its dense counterpart. Electrical failure of PZT95/5 ferroelectric ceramics under shock wave was found to be an extrinsic behavior and a tentative explanation was proposed based on the mechanical compressive stress-induced cracks initiation by epoxy resin.
Xian Lin,Jinling Chen,Gengsheng Xiao,Yujuan Xu,Daobang Tang,Jijun Wu,Jing Wen,Weidong Chen 한국식품과학회 2016 Food Science and Biotechnology Vol.25 No.3
Ultrasonic-microwave synergistic extraction (UMSE) was optimized for the extraction of oligosaccharides from longan pulp (OLP). Box-Behnken design was used to evaluate the effects of temperature (35-55oC), ultrasonic time (5-25 min), and water to material ratio (10-30 mL/g) on the extraction efficiency of crude OLP. A regression model was developed and its validity was statistically demonstrated. Significant interaction between temperature and water to material ratio was observed. The following optimal conditions for the extraction yield of crude OLP were determined: extraction temperature 55oC, ultrasonic time 18.52 min, and water to material ratio 10 mL/g. The extracted OLP were purified for the determination of molecular weight distribution and antioxidant activity. Results of matrix-assisted laser desorption ionization time-of-flight mass spectrometry revealed that the molecular weight distribution of the purified OLP ranged from m/z 495.138 to 795.511. The purified OLP exhibited a dose-dependent behavior in 1,1-diphenyl-2-picrylhydrazyl radical scavenging activity.