[1]郑昆文 李伟 江瑶 任禹衡 沈涛 张雯.运用高通量测序方法确定云南省2个家系及散发病例中的单纯型遗传性痉挛性截瘫的致病基因及发生模式[J].卒中与神经疾病杂志,2019,26(06):731-736.[doi:10.3969/j.issn.1007-0478.2019.06.022]
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运用高通量测序方法确定云南省2个家系及散发病例中的单纯型遗传性痉挛性截瘫的致病基因及发生模式()
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《卒中与神经疾病》杂志[ISSN:1007-0478/CN:42-1402/R]

卷:
第26卷
期数:
2019年06期
页码:
731-736
栏目:
论 著
出版日期:
2019-12-25

文章信息/Info

文章编号:
1007-0478(2019)06-0731-06
作者:
郑昆文 李伟 江瑶 任禹衡 沈涛 张雯
650032 昆明,云南省第一人民医院神经内科(郑昆文 李伟 江瑶 任禹衡),临床基础医学研究所[沈涛 张雯(通信作者)]
关键词:
单纯型遗传性痉挛性截瘫 家系 高通量测序 疾病发生模式
分类号:
R741
DOI:
10.3969/j.issn.1007-0478.2019.06.022
文献标志码:
A
摘要:
目的 运用高通量测序方法在云南省的2个家系和散发病例人群中探讨单纯型遗传性痉挛性截瘫(HSP)的发生模式,以期筛选出病理候选基因,探讨其遗传发生模式。方法 提取云南省2个家系和散发样本的全基因组DNA; 从家系样品中选择3例外送进行全外显子高通量测序; 依据筛选获得的候选突变基因,借助Sanger测序在剩余的家系样本和散发病例中进行验证,从而整合出这些样本的单纯型HSP疾病的遗传发生模式。结果 借助全外显子高通量测序分析和Sanger测序技术,在2个家系和2例散发病例中验证筛选,获得了13个候选致病突变基因。携带突变基因的异同不但是家系成员和散发病例患单纯型HSP疾病的互作成因,也使得不同家系呈现出不同的遗传模式。结论 结合高通量测序技术可以针对遗传性痉挛性截瘫这类疾病进行最大程度的广谱测序,从而获得有指导意义的突变基因,更好地确定疾病的家族遗传模式,最终作为后续疾病诊断和治疗的有效靶点服务于临床诊疗。

参考文献/References:


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备注/Memo

备注/Memo:
基金项目:云南省科技厅应用基础研究计划(昆医联合专项)(编号为2014FB086)(2019-02-21 收稿)
更新日期/Last Update: 2019-12-25