• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

对塞浦路斯遗传性心肌病患者进行大规模并行 DNA 测序,并提出双基因或寡基因遗传的假说。

Massive Parallel DNA Sequencing of Patients with Inherited Cardiomyopathies in Cyprus and Suggestion of Digenic or Oligogenic Inheritance.

机构信息

Molecular Medicine Research Center, biobank.cy Center of Excellence in Biobanking and Biomedical Research, University of Cyprus, Nicosia 2109, Cyprus.

Department of Cardiology, Nicosia General Hospital, Nicosia 2029, Cyprus.

出版信息

Genes (Basel). 2024 Feb 28;15(3):319. doi: 10.3390/genes15030319.

DOI:10.3390/genes15030319
PMID:38540378
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10970479/
Abstract

Inherited cardiomyopathies represent a highly heterogeneous group of cardiac diseases. DNA variants in genes expressed in cardiomyocytes cause a diverse spectrum of cardiomyopathies, ultimately leading to heart failure, arrythmias, and sudden cardiac death. We applied massive parallel DNA sequencing using a 72-gene panel for studying inherited cardiomyopathies. We report on variants in 25 families, where pathogenicity was predicted by different computational approaches, databases, and an in-house filtering analysis. All variants were validated using Sanger sequencing. Familial segregation was tested when possible. We identified 41 different variants in 26 genes. Analytically, we identified fifteen variants previously reported in the Human Gene Mutation Database: twelve mentioned as disease-causing mutations (DM) and three as probable disease-causing mutations (DM?). Additionally, we identified 26 novel variants. We classified the forty-one variants as follows: twenty-eight (68.3%) as variants of uncertain significance, eight (19.5%) as likely pathogenic, and five (12.2%) as pathogenic. We genetically characterized families with a cardiac phenotype. The genetic heterogeneity and the multiplicity of candidate variants are making a definite molecular diagnosis challenging, especially when there is a suspicion of incomplete penetrance or digenic-oligogenic inheritance. This is the first systematic study of inherited cardiac conditions in Cyprus, enabling us to develop a genetic baseline and precision cardiology.

摘要

遗传性心肌病是一组高度异质性的心脏疾病。在心肌细胞中表达的基因中的 DNA 变异导致了多种心肌病,最终导致心力衰竭、心律失常和心脏性猝死。我们应用大规模平行 DNA 测序技术,使用 72 个基因进行遗传性心肌病的研究。我们报告了 25 个家族的变异情况,通过不同的计算方法、数据库和内部过滤分析预测了其致病性。所有变异均通过 Sanger 测序进行验证。在可能的情况下,我们测试了家族遗传。我们在 26 个基因中发现了 41 个不同的变异。分析表明,我们在人类基因突变数据库中发现了 15 个先前报道的变异:12 个被认为是致病突变(DM),3 个被认为是可能的致病突变(DM?)。此外,我们还发现了 26 个新的变异。我们将这 41 个变异分类如下:28 个(68.3%)为意义不明的变异,8 个(19.5%)为可能致病的变异,5 个(12.2%)为致病性的变异。我们对具有心脏表型的家族进行了基因特征分析。遗传异质性和候选变异的多样性使得明确的分子诊断具有挑战性,特别是当怀疑不完全外显率或双基因/寡基因遗传时。这是塞浦路斯首次对遗传性心脏疾病进行的系统研究,使我们能够建立遗传基线和精准心脏病学。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cc3/10970479/000f6cabb378/genes-15-00319-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cc3/10970479/e24473e36e2c/genes-15-00319-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cc3/10970479/7eb8acc43c7e/genes-15-00319-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cc3/10970479/e5fee095eb83/genes-15-00319-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cc3/10970479/ce48abc933d2/genes-15-00319-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cc3/10970479/000f6cabb378/genes-15-00319-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cc3/10970479/e24473e36e2c/genes-15-00319-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cc3/10970479/7eb8acc43c7e/genes-15-00319-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cc3/10970479/e5fee095eb83/genes-15-00319-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cc3/10970479/ce48abc933d2/genes-15-00319-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cc3/10970479/000f6cabb378/genes-15-00319-g005.jpg

相似文献

1
Massive Parallel DNA Sequencing of Patients with Inherited Cardiomyopathies in Cyprus and Suggestion of Digenic or Oligogenic Inheritance.对塞浦路斯遗传性心肌病患者进行大规模并行 DNA 测序,并提出双基因或寡基因遗传的假说。
Genes (Basel). 2024 Feb 28;15(3):319. doi: 10.3390/genes15030319.
2
De novo and inherited variants in coding and regulatory regions in genetic cardiomyopathies.遗传性和新生编码及调控区变异与遗传性心肌病。
Hum Genomics. 2022 Nov 10;16(1):55. doi: 10.1186/s40246-022-00420-0.
3
Targeted 46-gene and clinical exome sequencing for mutations causing cardiomyopathies.针对导致心肌病的突变进行的46基因和临床外显子组测序。
Mol Cell Probes. 2015 Oct;29(5):308-14. doi: 10.1016/j.mcp.2015.05.004. Epub 2015 May 12.
4
Molecular Diagnosis of Inherited Cardiac Diseases in the Era of Next-Generation Sequencing: A Single Center's Experience Over 5 Years.遗传性心脏病的分子诊断:5 年单中心经验。
Mol Diagn Ther. 2021 May;25(3):373-385. doi: 10.1007/s40291-021-00530-w. Epub 2021 May 5.
5
Molecular analysis of inherited cardiomyopathy using next generation semiconductor sequencing technologies.使用下一代半导体测序技术进行遗传性心肌病的分子分析。
J Transl Med. 2018 Aug 30;16(1):241. doi: 10.1186/s12967-018-1605-5.
6
Genetic Insights from Consanguineous Cardiomyopathy Families.同型婚配心肌病家系的遗传学研究
Genes (Basel). 2023 Jan 10;14(1):182. doi: 10.3390/genes14010182.
7
Oligogenic Inheritance Underlying Incomplete Penetrance of Mutations in Hypogonadotropic Hypogonadism.低促性腺激素性性腺功能减退症中突变不完全外显背后的寡基因遗传
Front Genet. 2021 Sep 3;12:665174. doi: 10.3389/fgene.2021.665174. eCollection 2021.
8
Integrated clinical and omics approach to rare diseases: novel genes and oligogenic inheritance in holoprosencephaly.综合临床和组学方法研究罕见病:前脑无裂畸形的新基因和寡基因遗传。
Brain. 2019 Jan 1;142(1):35-49. doi: 10.1093/brain/awy290.
9
Novel variants identified with next-generation sequencing in Polish patients with cone-rod dystrophy.在波兰视锥-视杆营养不良患者中通过下一代测序鉴定出的新型变异体。
Mol Vis. 2018 Apr 26;24:326-339. eCollection 2018.
10
Navigating the penetrance and phenotypic spectrum of inherited cardiomyopathies.遗传性心肌病的外显率和表型谱分析。
Heart Fail Rev. 2024 Sep;29(5):873-881. doi: 10.1007/s10741-024-10405-x. Epub 2024 Jun 19.

本文引用的文献

1
Twelve years of SAMtools and BCFtools.SAMtools 和 BCFtools 十二年。
Gigascience. 2021 Feb 16;10(2). doi: 10.1093/gigascience/giab008.
2
The Human Gene Mutation Database (HGMD): optimizing its use in a clinical diagnostic or research setting.人类基因突变数据库(HGMD):优化其在临床诊断或研究环境中的使用。
Hum Genet. 2020 Oct;139(10):1197-1207. doi: 10.1007/s00439-020-02199-3. Epub 2020 Jun 28.
3
Integrated Informatics Analysis of Cancer-Related Variants.癌症相关变异的综合信息学分析
JCO Clin Cancer Inform. 2020 Mar;4:310-317. doi: 10.1200/CCI.19.00132.
4
Hypertrophic Cardiomyopathy: An Overview of Genetics and Management.肥厚型心肌病:遗传学与管理概述。
Biomolecules. 2019 Dec 16;9(12):878. doi: 10.3390/biom9120878.
5
The REDCap consortium: Building an international community of software platform partners.REDCap 联盟:构建软件平台合作伙伴的国际社区。
J Biomed Inform. 2019 Jul;95:103208. doi: 10.1016/j.jbi.2019.103208. Epub 2019 May 9.
6
Epidemiology of ATTRV30M neuropathy in Cyprus and the modifier effect of complement C1q on the age of disease onset.ATTRV30M 神经病在塞浦路斯的流行病学和补体 C1q 对疾病发病年龄的修饰作用。
Amyloid. 2018 Dec;25(4):220-226. doi: 10.1080/13506129.2018.1534731. Epub 2018 Dec 20.
7
Digenic inheritance and genetic modifiers.双基因遗传和遗传修饰物。
Clin Genet. 2018 Mar;93(3):429-438. doi: 10.1111/cge.13150. Epub 2018 Jan 25.
8
Arrhythmogenic cardiomyopathy: pathology, genetics, and concepts in pathogenesis.致心律失常性右室心肌病:病理学、遗传学及发病机制相关概念。
Cardiovasc Res. 2017 Oct 1;113(12):1521-1531. doi: 10.1093/cvr/cvx150.
9
Dilated Cardiomyopathy: Genetic Determinants and Mechanisms.扩张型心肌病:遗传决定因素与机制
Circ Res. 2017 Sep 15;121(7):731-748. doi: 10.1161/CIRCRESAHA.116.309396.
10
Novel Genetic Triggers and Genotype-Phenotype Correlations in Patients With Left Ventricular Noncompaction.左心室心肌致密化不全患者的新型遗传触发因素及基因型-表型相关性
Circ Cardiovasc Genet. 2017 Aug;10(4). doi: 10.1161/CIRCGENETICS.117.001763.