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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.

DOI:10.1007/s10048-025-00824-9
PMID:40388045
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。

相似文献

1
Identification of mutations in five Pakistani families with Epilepsy.五个巴基斯坦癫痫家族中突变的鉴定。
Neurogenetics. 2025 May 19;26(1):44. doi: 10.1007/s10048-025-00824-9.
2
Next-generation sequencing to genetically diagnose a diverse range of inherited eye disorders in 15 consanguineous families from Pakistan.利用下一代测序技术对来自巴基斯坦的 15 个近亲家族的多种遗传性眼部疾病进行基因诊断。
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Syndromic forms of inherited retinal dystrophies: a comprehensive molecular diagnosis of consanguineous Pakistani families using capture panel sequencing.遗传性视网膜营养不良的综合征形式:使用捕获面板测序对巴基斯坦近亲家庭进行全面分子诊断。
Mol Vis. 2025 Mar 26;31:69-83. eCollection 2025.
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Molecular genetics, neuroimaging outcomes, and structural analyses of novel and recurrent variants of WDR62 gene in two consanguineous Pakistani families with autosomal recessive primary microcephaly.在两个有血缘关系的巴基斯坦原发性小头畸形常染色体隐性家族中,对 WDR62 基因的新型和复发性变异进行分子遗传学、神经影像学结果和结构分析。
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Exome Sequencing of Consanguineous Pashtun Families With Familial Epilepsy Reveals Causative and Candidate Variants in TSEN54, MOCS2, and OPHN1.常染色体隐性遗传家系性癫痫患者外显子组测序揭示 TSEN54、MOCS2 和 OPHN1 中的致病和候选变异。
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Phenotypic and Genetic Heterogeneity of a Pakistani Cohort of 15 Consanguineous Families Segregating Variants in Leber Congenital Amaurosis-Associated Genes.一个巴基斯坦近亲结婚家庭队列中15个家庭的表型和基因异质性,这些家庭在莱伯先天性黑蒙相关基因中存在分离变异。
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Previously defined variants of uncertain significance may play an important role in epilepsy and interactions between certain variants may become pathogenic.先前定义的意义未明的变异可能在癫痫中起重要作用,并且某些变异之间的相互作用可能会变得具有致病性。
Epilepsia Open. 2024 Dec;9(6):2443-2453. doi: 10.1002/epi4.13085. Epub 2024 Nov 7.
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Genetic Spectrum of Syndromic and Non-Syndromic Hearing Loss in Pakistani Families.巴基斯坦家族性综合征型和非综合征型听力损失的遗传谱。
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Global genetic insight contributed by consanguineous Pakistani families segregating hearing loss.巴基斯坦近亲家族遗传性听力损失的全球遗传学研究。
Hum Mutat. 2019 Jan;40(1):53-72. doi: 10.1002/humu.23666. Epub 2018 Nov 18.

本文引用的文献

1
Rett Syndrome and Duplication Syndrome: Disorders of MeCP2 Dosage.雷特综合征与重复综合征:MeCP2剂量紊乱疾病
Neuropsychiatr Dis Treat. 2022 Nov 29;18:2813-2835. doi: 10.2147/NDT.S371483. eCollection 2022.
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Epilepsy Behav Rep. 2020 Oct 14;15:100391. doi: 10.1016/j.ebr.2020.100391. eCollection 2021.
3
Recent advances in gene therapy for neurodevelopmental disorders with epilepsy.用于治疗伴有癫痫的神经发育障碍的基因治疗的最新进展。
J Neurochem. 2021 Apr;157(2):229-262. doi: 10.1111/jnc.15168. Epub 2020 Sep 28.
4
Advances in understanding of Rett syndrome and MECP2 duplication syndrome: prospects for future therapies.对雷特综合征和 MECP2 重复综合征的认识进展:未来治疗的前景。
Lancet Neurol. 2020 Aug;19(8):689-698. doi: 10.1016/S1474-4422(20)30217-9.
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Sodium channel epilepsies and neurodevelopmental disorders: from disease mechanisms to clinical application.钠离子通道癫痫与神经发育障碍:从疾病机制到临床应用。
Dev Med Child Neurol. 2020 Jul;62(7):784-792. doi: 10.1111/dmcn.14519. Epub 2020 Mar 30.
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Lafora Disease: Report of a Rare Entity.拉福拉病:一种罕见病症的报告。
Cureus. 2020 Jan 28;12(1):e6793. doi: 10.7759/cureus.6793.
7
The global burden of epilepsy report: Implications for low- and middle-income countries.全球癫痫负担报告:对中低收入国家的影响。
Epilepsy Behav. 2020 Apr;105:106949. doi: 10.1016/j.yebeh.2020.106949. Epub 2020 Feb 20.
8
Clinical spectrum of -related epileptic disorders.与相关的癫痫性疾病的临床谱。
Neurology. 2019 Mar 12;92(11):e1238-e1249. doi: 10.1212/WNL.0000000000007089. Epub 2019 Feb 8.
9
The Epidemiological Characteristics of Epilepsy in the Province of Khyber Pakhtunkhwa, Pakistan.巴基斯坦开伯尔-普赫图赫瓦省癫痫的流行病学特征
Front Neurol. 2018 Nov 6;9:845. doi: 10.3389/fneur.2018.00845. eCollection 2018.
10
Epilepsy-associated genes.癫痫相关基因
Seizure. 2017 Jan;44:11-20. doi: 10.1016/j.seizure.2016.11.030. Epub 2016 Dec 6.