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五个巴基斯坦癫痫家族中突变的鉴定。

Identification of mutations in five Pakistani families with Epilepsy.

作者信息

Ahsan Nayab, Ahmad Arsalan, Hussain Shahnawaz, Fatima Nasreen, Yousafzai Imran Khan, Kalsoom Umm-E-, Dad Rubina, Hassan Muhammad Jawad

机构信息

NUMS Department of Biological Sciences, National University of Medical Sciences, Rawalpindi, Pakistan.

Division of Neurology, Shifa International Hospitals, Shifa Tameer e Millat University, Islamabad, Pakistan.

出版信息

Neurogenetics. 2025 May 19;26(1):44. doi: 10.1007/s10048-025-00824-9.

Abstract

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.

摘要

癫痫是一组以癫痫发作作为特征的神经系统疾病,癫痫发作的表现形式多样,从短暂且几乎难以察觉的发作到长时间剧烈抽搐不等。由于巴基斯坦人群近亲结婚(血缘关系)比例较高,因此很可能会出现有多个个体受影响且呈现出新型或已知癫痫表型的家庭。本研究旨在确定特定家庭中癫痫病症(孤立性或综合征性)的遗传病因。为此,在经过全面临床调查后,招募了五个有多个个体受影响且表现出某种癫痫形式的家庭(A - E)。应用基于二代测序(NGS)的基因 panel 检测来确定这些家庭中的致病变异。每个家庭选取一名受影响个体的 DNA 样本,送往一家知名基因检测实验室(美国 Invitae)进行癫痫综合基因 panel 检测。使用生物信息学工具(SIFT、PolyPhen2)来验证所鉴定突变的致病性。我们在这五个家庭中鉴定出五个先前报道过的突变;通过生物信息学分析,所有这些突变均被预测为致病性突变。这些发现无疑将有助于增进我们对遗传性癫痫病因的理解,并有助于这些家庭的遗传咨询和临床管理。

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