. Author manuscript; available in PMC: 2015 Jun 30.

Published in final edited form as: J Am Coll Cardiol. 2014 Aug 26;64(8):832–839. doi: 10.1016/j.jacc.2014.04.073

TABLE 2.

Transgenic Mouse Models That Include Bicuspid Aortic Valve

Allele	Penetrance	Other Phenotypes	Ref. #
Gata5^tm1.1Nemr	7/28	LVH	(47)
Tg(GATA5-cre)^1Krc/Acvr1^tm1Vk	3/22	Perimembranous VSDs	(59)
Nos3^tm1Unc	5/12	None	(60)
Hoxa1^tm3Mrc	4/17	IAA, VSD	(61)
Tbx1^tm1Pa	5/34	IAA, TOF, coronary anomalies	(62)
Hoxa3^tm1(cre)Moon/Fgf8^tm2Moon	7/33	IAA, coronary anomalies	(63)
Nkx2–5^tm1(cre)Rjs/Frs2^tm1Fwan	20%	OA, DORV	(64)
Nkx2–5^tm1Wehi^* Nkx2–5^tm4Rph^*	11/100 2/98	Conduction defects, PFO	(65)

Allele information is taken from Mouse Genome Informatics (http://www.informatics.jax.org).

Heterozygotes.

DORV = double-outlet right ventricle; IAA = interrupted aortic arch; LVH = left ventricular hypertrophy; OA = overriding aorta; PFO = patent foramen ovale; TOF = tetralogy of Fallot; VSD = ventricular septal defect.