Chen Bo, Hou Aiping, Zhao Lin, Liu Ying, Shi Xin, Du Bowen, Yu Yu, Zhao Pengjun, Gao Ying
Department of Pediatric Cardiology, Xin Hua Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Department of Pediatric, Shidong Hospital, Shanghai, China.
Front Genet. 2020 Nov 12;11:600787. doi: 10.3389/fgene.2020.600787. eCollection 2020.
Patent ductus arteriosus (PDA) is a common congenital cardiovascular malformation with both inherited and acquired causes. Several genes have been reported to be related to PDA, but the molecular pathogenesis is still unclear. Here, we screened a population matched cohort of 39 patients with PDA and 100 healthy children using whole exome sequencing (WES). And identified 10 copy number variants (CNVs) and 20 candidate genes using Gene ontology (GO) functional enrichment analysis. In gene network analysis, we screened 7 pathogenic CNVs of 10 candidate genes (MAP3K1, MYC, VAV2, WDR5, RXRA, APLNR, TJP1, ERCC2, FOSB, CHRNA4). Further analysis of transcriptome array showed that 7 candidate genes (MAP3K1, MYC, VAV2, APLNR, TJP1, FOSB, CHRNA4) were indeed significantly expressed in human embryonic heart. Moreover, CHRNA4 was observed the most important genes. Our data provided rare CNVs as potential genetic cause of PDA in humans and also advance understanding of the genetic components of PDA.
动脉导管未闭(PDA)是一种常见的先天性心血管畸形,有遗传和后天多种病因。已有多个基因被报道与PDA相关,但分子发病机制仍不清楚。在此,我们使用全外显子组测序(WES)对39例PDA患者和100名健康儿童组成的匹配队列进行了筛查。并通过基因本体(GO)功能富集分析鉴定出10个拷贝数变异(CNV)和20个候选基因。在基因网络分析中,我们从10个候选基因(MAP3K1、MYC、VAV2、WDR5、RXRA、APLNR、TJP1、ERCC2、FOSB、CHRNA4)中筛选出7个致病CNV。转录组阵列的进一步分析表明,7个候选基因(MAP3K1、MYC、VAV2、APLNR、TJP1、FOSB、CHRNA4)在人类胚胎心脏中确实有显著表达。此外,CHRNA4被认为是最重要的基因。我们的数据提供了罕见的CNV作为人类PDA的潜在遗传病因,也增进了对PDA遗传成分的理解。