Fate mapping of Trps1 daughter cells during cardiac development using novel Trps1-Cre mice.
Fate mapping of Trps1 daughter cells during cardiac development using novel Trps1-Cre mice.
复制标题
使用新型 Trps1-Cre 小鼠绘制心脏发育过程中 Trps1 子细胞的命运图谱。
作者：
Nomir AG;Takeuchi Y;Fujikawa J;El Sharaby AA;Wakisaka S;Abe M
Tricho - rhino - phalangal syndrome （TRPS）是一种罕见的先天性疾病，以毛发生长异常和骨骼畸形为特征。这些导致头发稀疏，身材矮小，和早期发作的关节问题。最近的报告显示，相对较高比例的TRPS患者表现出广泛的先天性心脏缺陷。为了确定Trps1在体内的转录调控，我们培育了新的转基因小鼠，在小鼠Trps1近端启动子序列（Trps1‐Cre）下表达Cre重组酶。我们将这些小鼠与Cre报告小鼠杂交以鉴定Trps1子细胞。阑尾关节组织、毛囊真皮乳头、心瓣膜、主动脉窦、心房壁、室间隔均可见标记细胞。原位分析显示trps1表达受限，在流出道心内膜垫和所有成熟心脏瓣膜的小叶中均可见。这些结果表明，Trps1近端启动子序列包含一些组织特异性Trps1调控区域。此外，我们的研究结果部分解释了为什么TRPS患者表现出广泛的先天性心脏缺陷，尽管trps1的表达在更有限的情况下被观察到。创世纪54:37 79 - 388,2016。©2016 Wiley期刊公司
Tricho‐rhino‐phalangeal syndrome (TRPS) is a rare congenital disorder that is characterized by abnormal hair growth and skeletal deformities. These result in sparse hair, short stature, and early onset of joint problems. Recent reports have shown that a relatively high proportion of patients with TRPS exhibit a broad range of congenital heart defects. To determine the regulation of Trps1 transcriptionin vivo, we generated novel transgenic mice, which expressed Cre recombinase under the murine Trps1 proximal promoter sequence (Trps1‐Cre). We crossed these mice with Cre reporter mice to identify Trps1 daughter cells. Labeled cells were observed in the appendicular joint tissue, dermal papilla of the hair follicles, cardiac valves, aortic sinus, atrial walls, and the interventricular septum.In situanalysis showed restrictedTrps1expression, which was observed in endocardial cushions of the outflow tract, and in leaflets of all mature cardiac valves. These results suggest that the Trps1 proximal promoter sequence contains some of the tissue‐specific Trps1 regulatory region. Further, our findings partially explain why patients with TRPS show a broad range of congenital cardiac defects, althoughTrps1expression is observed in a more restricted fashion. genesis 54:379–388, 2016. © 2016 Wiley Periodicals, Inc.