Decreased endothelial cell retinoic acid signaling accelerates progression of single ventricle pulmonary arteriovenous malformations

To investigate transcriptomic changes in the endothelial cells of a mouse model of JP/HHT (Smad4), endothelial cells were isolated from the lungs of P5 Smad4 control (Smad4 conditional floxed mice; Smad4 f/f) and Smad4 HHT mice (Smad4 f/f;Cdh5-CreERT2). Data sets were compared to reveal differentially expressed genes that may provide clues into the molecular changes driving HHT phenotypes. Overall design: RNA sequencing of isolated lung endothelial cells from Smad4 endothelial cell-specific knock-out mouse mutants and respective floxed controls.