结节性硬化
TSC1
产前诊断
错义突变
遗传学
先证者
遗传咨询
无义突变
桑格测序
突变
生物
TSC2
外显子
基因
医学
胎儿
怀孕
病理
细胞凋亡
PI3K/AKT/mTOR通路
作者
Yuchun Pan,Weiqing Wu,Caiqun Luo,Jiansheng Xie,Zhiyong Xu,Geng Qian,Hao Ying
出处
期刊:PubMed
[National Institutes of Health]
日期:2018-02-10
卷期号:35 (1): 18-22
标识
DOI:10.3760/cma.j.issn.1003-9406.2018.01.004
摘要
OBJECTIVE To provide prenatal diagnosis for families affected with tuberous sclerosis complex and explore the correlation between phenotype and genotype. METHODS For probands from 10 families, all exons and splicing regions of the TSC1 and TSC2 genes were analyzed with high throughput DNA sequencing. Suspected mutations were verified by Sanger sequencing. RESULTS All probands were found to have mutations, which included 1 case with TSC1 mutation and 9 cases with TSC2 mutations (missense mutations in 6, nonsense mutations in 2, and frameshifting mutation in 1 case). Prenatal diagnosis was provided for 9 cases, and 1 fetus was found to carry a mutation. Genetic analysis has identified a novel pathogenic mutation (TSC2 c.2415-2416 ins GT). CONCLUSION Identification of pathological mutations for tuberous sclerosis complex can facilitate genetic counseling and prenatal diagnosis for the affected families.
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