Genetic Variants Identified from Epilepsy of Unknown Etiology in Chinese Children by Targeted Exome Sequencing

被引:55
作者
Wang, Yimin [1 ]
Du, Xiaonan [1 ]
Bin, Rao [2 ]
Yu, Shanshan [2 ]
Xia, Zhezhi [3 ]
Zheng, Guo [4 ]
Zhong, Jianmin [5 ]
Zhang, Yunjian [1 ]
Jiang, Yong-hui [6 ,7 ,8 ]
Wang, Yi [1 ,9 ]
机构
[1] Fudan Univ, Childrens Hosp, Div Neurol, 399 Wanyuan Rd, Shanghai 201102, Peoples R China
[2] BGI, Shenzhen 518083, Peoples R China
[3] Zhe Jiang Childrens Hosp, 3333 Binsheng Rd, Hangzhou, Zhejiang, Peoples R China
[4] Nan Jing Childrens Hosp, 72 Guangzhou Rd, Nanjing, Jiangsu, Peoples R China
[5] Jiangxi Childrens Hosp, 122 Yangming Rd, Nanchang, Jiangxi, Peoples R China
[6] Duke Univ, Sch Med, Dept Pediat, Div Med Genet, 905 S LaSalle ST, Durham, NC 27708 USA
[7] Duke Univ, Univ Genom & Genet Program, 905 S LaSalle ST, Durham, NC 27708 USA
[8] Duke Univ, Sch Med, Dept Neurobiol, 905 S LaSalle ST, Durham, NC 27708 USA
[9] Fudan Univ, Inst Brain Sci, 138 Yi Xue Yuan Rd, Shanghai 200032, Peoples R China
来源
SCIENTIFIC REPORTS | 2017年 / 7卷
基金
中国国家自然科学基金;
关键词
GABA(A) RECEPTOR SUBUNITS; FEBRILE SEIZURES PLUS; DIAGNOSTIC YIELD; CHD2; MUTATIONS; RARE CAUSE; SPECTRUM; ENCEPHALOPATHIES; PHENOTYPES; MODEL; PRRT2;
D O I
10.1038/srep40319
中图分类号
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号
07 ; 0710 ; 09 ;
摘要
Genetic factors play a major role in the etiology of epilepsy disorders. Recent genomics studies using next generation sequencing (NGS) technique have identified a large number of genetic variants including copy number (CNV) and single nucleotide variant (SNV) in a small set of genes from individuals with epilepsy. These discoveries have contributed significantly to evaluate the etiology of epilepsy in clinic and lay the foundation to develop molecular specific treatment. However, the molecular basis for a majority of epilepsy patients remains elusive, and furthermore, most of these studies have been conducted in Caucasian children. Here we conducted a targeted exome-sequencing of 63 trios of Chinese epilepsy families using a custom-designed NGS panel that covers 412 known and candidate genes for epilepsy. We identified pathogenic and likely pathogenic variants in 15 of 63 (23.8%) families in known epilepsy genes including SCN1A, CDKL5, STXBP1, CHD2, SCN3A, SCN9A, TSC2, MBD5, POLG and EFHC1. More importantly, we identified likely pathologic variants in several novel candidate genes such as GABRE, MYH1, and CLCN6. Our results provide the evidence supporting the application of custom-designed NGS panel in clinic and indicate a conserved genetic susceptibility for epilepsy between Chinese and Caucasian children.
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页数:10
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