期刊
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
卷 22, 期 24, 页码 -出版社
MDPI
DOI: 10.3390/ijms222413270
关键词
great arteries transposition; endothelial cells; angiogenesis; Notch signaling pathway; iPSC
资金
- RETICS Program rom Instituto de Salud Carlos III [RD16/0011/0004]
- FEDER 'una manera de hacer Europa'
- National Institutes of Health [R01 HL130020, R01 HL145676, R01 HL141851]
- BHF Centre [RM/17/1/33377]
- Instituto de Investigacion Sanitaria La Fe [2014/0151]
- Conselleria de Sanitat Universal i Salut Publica [ACIF/2018/259]
- European Union through the Operational Programme European Regional Development Fund (FEDER) of the Valencian Community 2014-2020
- Medical Research Council [MR/R025002/1]
- NIHR Imperial Biomedical Research Centre
- Hungarian National Research, Development and Innovation Fund [2020-1.1.6-2021-00013, K128369]
- MRC [MR/R025002/1] Funding Source: UKRI
The study established an iPSC-based cellular model to investigate the etiology of d-TGA, finding that iPSC-ECs from patients with d-TGA showed impaired ability to develop tubular structures in an in vitro capillary-like tube formation assay. Interactome studies revealed downregulation of biological processes related to Notch signaling, circulatory system development and angiogenesis in d-TGA patients.
The dextro-transposition of the great arteries (d-TGA) is one of the most common congenital heart diseases. To identify biological processes that could be related to the development of d-TGA, we established induced pluripotent stem cell (iPSC) lines from two patients with d-TGA and from two healthy subjects (as controls) and differentiated them into endothelial cells (iPSC-ECs). iPSC-EC transcriptome profiling and bioinformatics analysis revealed differences in the expression level of genes involved in circulatory system and animal organ development. iPSC-ECs from patients with d-TGA showed impaired ability to develop tubular structures in an in vitro capillary-like tube formation assay, and interactome studies revealed downregulation of biological processes related to Notch signaling, circulatory system development and angiogenesis, pointing to alterations in vascular structure development. Our study provides an iPSC-based cellular model to investigate the etiology of d-TGA.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据