More recently, Jordan et al. (129) reported that Frem2ne/ne and Fras1Q1263*/Q1263* mice developed an almost identical type of anterior midline CDH with herniated viscera covered by a thin membranous sac as seen in Frem1-defcient mice, thus concluding that loss of the Frem1/Frem2/Fras1 complex or its function results in retrosternal CDH in these animals. Here, FREM1 is linked to congenital diaphragmatic hernia.