Table 2.

Endocardial cushion phenotypes in mouse models harboring mutations in Notch-signaling mediators

Target gene	Model	Endocardial cushion phenotype	References
Hey1, HeyL	Global double knockout	Impaired EMT in E9.5 AV canal explant cultures	Fischer et al. 2007
Hey2	Predicted null allele (Hey2lacz)	EMT occurs, but cushion maturation is impaired	Donovan et al. 2002
Jag1	VE-cadherin-Cre deletion	Hypoplastic endocardial cushions at E10.5, temporarily delayed EMT	Hofmann et al. 2012
MAML	Dominant negative (dn), Isl1-Cre deletion	Hypocellular OFT endocardial cushions at E10.5	High et al. 2009
MAML	Tet-inducible dn under control of VE-cadherin promoter	Tet-treatment at E10.5, impaired EMT at E11.5 and E12.5	Chang et al. 2014
NICD1	Mesp1-Cre transgenic	Ectopic cell masses in endocardial cushions at E9.5	Watanabe et al. 2006
Notch1	Global knockout	Impaired EMT in E9.5 AV canal explant cultures	Timmerman et al. 2004
	VE-cadherin-Cre deletion	Delayed EMT at E10.5	Hofmann et al. 2012
RBPjk	Global knockout	EMT is initiated but endocardial cushions lack mesenchyme cells at E9.5	Timmerman et al. 2004

(EMT) endothelial-to-mesenchyme transformation, (OFT) outflow tract, (AV) atrioventricular.