GATA-6 regulates semaphorin 3C and is required in cardiac neural crest for cardiovascular morphogenesis
J. Clin. Invest. John J. Lepore, et al. 116:929 doi:10.1172/JCI27363 [
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Figure 2Cardiovascular abnormalities produced by conditional
GATA-6 deletion. Aortic arch patterning and cardiac outflow tract septation were examined in E18.5
GATA-6F/F(Cre–;
A and
F–
I),
SMCre+GATA-6F/F (SMCre+;
B,
C, and
K–
N), and
Wnt1Cre+GATA-6 (WCre+;
D, E,
J, and
O) embryos using polymer vascular casting (
A–E) and H&E staining (
F–O). (
A) In normal aortic arch patterning, the ascending aorta (AAo) and pulmonary artery (PA) are distinct, septated vessels. The ductus arteriosus (DA) is patent and connects the PA to the proximal descending aorta (DAo). The right subclavian artery (RS) branches from the AAo. (
B) SMCre
+ embryo demonstrating truncus arteriosus (TA) and hypoplastic aortic arch (arch). (
C) SMCre
+ embryo demonstrating interrupted aortic arch (IAA). (
D) WCre
+ embryo demonstrating TA, hypoplastic arch, and retroesophageal right subclavian artery (RERS). (
E) WCre
+ embryo demonstrating IAA. (
F–
I) Serial histological sections through the heart and great vessels of a normal embryo. (
F) The DA connects the PA to the DAo. The RS branches from the AAo. (
G and
H) The AAo and PA are distinct, septated vessels. The pulmonary valve (PV) is shown. (
I) An intact ventricular septum (arrow) separates the right and left ventricles. (
K–
N) Corresponding serial sections from a SMCre
+ embryo
. (
K) A RERS branches from the DAo and travels posterior to the esophagus (E). (
L–
N) There is single outflow tract vessel, or TA; a single, common aorticopulmonary valve (APV); and a membranous ventricular septal defect (VSD). (
J and
O) Representative WntCre
+ embryo exhibiting TA and VSD. Original magnification, ×20 (
A–
E); ×40 (
F–
O).