Boogerd Cornelis J, Aneas Ivy, Sakabe Noboru, Dirschinger Ralph J, Cheng Quen J, Zhou Bin, Chen Ju, Nobrega Marcelo A, Evans Sylvia M
J Clin Invest. 2016 Aug 1;126(8):3023-35. doi: 10.1172/JCI85350. Epub 2016 Jun 27.
Mutations in the T-box transcription factor TBX20 are associated with multiple forms of congenital heart defects, including cardiac septal abnormalities, but our understanding of the contributions of endocardial TBX20 to heart development remains incomplete. Here, we investigated how TBX20 interacts with endocardial gene networks to drive the mesenchymal and myocardial movements that are essential for outflow tract and atrioventricular septation. Selective ablation of Tbx20 in murine endocardial lineages reduced the expression of extracellular matrix and cell migration genes that are critical for septation. Using the assay for transposase-accessible chromatin with high-throughput sequencing (ATAC-seq), we identified accessible chromatin within endocardial lineages and intersected these data with TBX20 ChIP-seq and chromatin loop maps to determine that TBX20 binds a conserved long-range enhancer to regulate versican (Vcan) expression. We also observed reduced Vcan expression in Tbx20-deficient mice, supporting a direct role for TBX20 in Vcan regulation. Further, we show that the Vcan enhancer drove reporter gene expression in endocardial lineages in a TBX20-binding site-dependent manner. This work illuminates gene networks that interact with TBX20 to orchestrate cardiac septation and provides insight into the chromatin landscape of endocardial lineages during septation.
T盒转录因子TBX20的突变与多种先天性心脏缺陷有关,包括心脏间隔异常,但我们对心内膜TBX20在心脏发育中的作用的理解仍不完整。在这里,我们研究了TBX20如何与心内膜基因网络相互作用,以驱动对流出道和房室间隔形成至关重要的间充质和心肌运动。在小鼠心内膜谱系中选择性敲除Tbx20会降低对间隔形成至关重要的细胞外基质和细胞迁移基因的表达。使用高通量测序转座酶可及染色质分析(ATAC-seq),我们确定了心内膜谱系内的可及染色质,并将这些数据与TBX20染色质免疫沉淀测序(ChIP-seq)和染色质环图谱相交,以确定TBX20结合一个保守的长程增强子来调节多功能蛋白聚糖(Vcan)的表达。我们还观察到Tbx20缺陷小鼠中Vcan表达降低,支持TBX20在Vcan调节中的直接作用。此外,我们表明Vcan增强子以TBX20结合位点依赖的方式驱动心内膜谱系中的报告基因表达。这项工作阐明了与TBX20相互作用以协调心脏间隔形成的基因网络,并提供了对间隔形成期间心内膜谱系染色质景观的见解。