五个巴基斯坦癫痫家族的突变鉴定。

IF 1.6 4区 医学 Q3 CLINICAL NEUROLOGY
Nayab Ahsan, Arsalan Ahmad, Shahnawaz Hussain, Nasreen Fatima, Imran Khan Yousafzai, Umm-E- Kalsoom, Rubina Dad, Muhammad Jawad Hassan
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引用次数: 0

摘要

癫痫是一组以癫痫发作为特征的神经系统疾病,其发作可能从短暂且几乎无法检测到长时间的剧烈颤抖不等。由于巴基斯坦人口中近亲婚姻(血亲关系)的比例很高,有多个受影响个体的家庭可能会出现新的或已知的癫痫表型。本研究的目的是在选定的家庭中确定癫痫状况(孤立或综合征)的遗传原因。为此目的,在彻底的临床调查后,招募了5个家庭(a - e),其中有多名患者表现出一种癫痫形式。应用基于下一代测序(NGS)的基因面板检测技术鉴定这些家族的致病变异。每个家庭一名受影响个体的DNA样本被送到著名的基因检测实验室(Invitae, USA)进行癫痫综合基因面板检测。使用生物信息学(SIFT, PolyPhen2)工具验证鉴定的突变的致病性。我们在这五个家族中确定了五个先前报道的突变;经生物信息学分析,预测均具有致病性。这一发现将有助于提高我们对遗传性癫痫病因的理解,并将促进这些家庭的遗传咨询和临床管理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Identification of mutations in five Pakistani families with Epilepsy.

Epilepsy is a group of neurological conditions characterized by epileptic seizures, which are episodes that may vary from brief and nearly undetectable to long periods of vigorous shaking. Due to high rates of close family marriages (consanguinity) in the Pakistani population, families with multiple affected individuals showing novel or known epilepsy phenotypes are likely to present. The present study aimed to identify the genetic causes of epileptic conditions (isolated or syndromic) in selected families. For this purpose, five families (A-E) with multiple affected individuals showing a form of epilepsy were recruited after thorough clinical investigations. Next Generation Sequencing (NGS) based gene panel testing was applied to identify the pathogenic variants in these families. DNA samples of one affected individual from each family were sent to a renowned genetic testing lab (Invitae, USA) for Epilepsy Comprehensive Gene Panel Testing. Bioinformatics (SIFT, PolyPhen2) tools were used to validate the pathogenicity of identified mutations. We identified five previously reported mutations in these five families; all of them were predicted to be pathogenic by bioinformatics analysis. The findings would certainly help enhance our understanding regarding the etiology of inherited epilepsies and would facilitate genetic counseling and clinical management in these families.

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来源期刊
Neurogenetics
Neurogenetics 医学-临床神经学
CiteScore
3.90
自引率
0.00%
发文量
24
审稿时长
6 months
期刊介绍: Neurogenetics publishes findings that contribute to a better understanding of the genetic basis of normal and abnormal function of the nervous system. Neurogenetic disorders are the main focus of the journal. Neurogenetics therefore includes findings in humans and other organisms that help understand neurological disease mechanisms and publishes papers from many different fields such as biophysics, cell biology, human genetics, neuroanatomy, neurochemistry, neurology, neuropathology, neurosurgery and psychiatry. All papers submitted to Neurogenetics should be of sufficient immediate importance to justify urgent publication. They should present new scientific results. Data merely confirming previously published findings are not acceptable.
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