Chen Hanying, Zhang Wenjun, Li Deqiang, Cordes Tim M, Mark Payne R, Shou Weinian
Riley Heart Research Center, Herman B. Wells Center for Pediatric Research, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN 46202, USA.
Pediatr Cardiol. 2009 Jul;30(5):626-34. doi: 10.1007/s00246-009-9406-5. Epub 2009 Apr 25.
Ventricular trabeculation and compaction are two of the many essential steps for generating a functionally competent ventricular wall. A significant reduction in trabeculation is usually associated with ventricular compact zone deficiencies (hypoplastic wall), which commonly lead to embryonic heart failure and early embryonic lethality. In contrast, hypertrabeculation and lack of ventricular wall compaction (noncompaction) are closely related defects in cardiac embryogenesis associated with left ventricular noncompaction, a genetically heterogeneous disorder. Here we summarize our recent findings through the analyses of several genetically engineered mouse models that have defects in cardiac trabeculation and compaction. Our data indicate that cellular growth and differentiation signaling pathways are keys in these ventricular morphogenetic events.
心室小梁形成和致密化是生成功能正常的心室壁的众多关键步骤中的两个。小梁形成的显著减少通常与心室致密区缺陷（发育不全的心室壁）相关，这通常会导致胚胎心力衰竭和早期胚胎致死。相反，小梁过度形成和心室壁致密化缺失（心肌致密化不全）是心脏胚胎发生中与左心室心肌致密化不全密切相关的缺陷，左心室心肌致密化不全是一种遗传异质性疾病。在此，我们通过对几种在心脏小梁形成和致密化方面存在缺陷的基因工程小鼠模型的分析，总结我们最近的研究结果。我们的数据表明，细胞生长和分化信号通路是这些心室形态发生事件的关键。
