Phenotype Image Detail

Image

Caption

Transverse sections of the heart of term wild-type (a,d and g), Ece1^tm1Reh/Ece1^tm1Reh (ECE-1^-/-) (b,e and h), and Ece1^tm1Reh/Ece1^tm1Reh Ece2^tm1Ywa/Ece2^tm1Ywa (ECE-1^-/-; ECE-2^-/-) (c,f and i) embryos stained with hematoxylin and eosin. Original magnification is x10. (a,d and g) At the level of the pulmonary trunk (P), the ascending aorta (As) is seen as posterior and right to the pulmonary trunk in wild-type embryos. The aortic outflow (Ao) comes from the left ventricle (LV) (d), and the left AV valve opens to the left ventricle (g). (b, e and h) In single mutant embryos, relative distance between the ascending aorta and the pulmonary trunk shortens, and the ascending aorta is seen anteriorly (b). Owing to the malalignment of outflow tracts, aortic and pulmonary tracts are seen in the same plane, originating from the right ventricle (RV) (DORV). Two great vessels do cross over (e). A small VSD is indicated by an arrow (h). (c,f and i) In double mutant embryos, the ascending aorta is seen further anteriorly, with the pulmonary trunk seen posteriorly to ascending aorta (a). Aortic and pulmonary outflows both originate from the right ventricle (DORV), and the two great vessels do not cross over. Note hypoplasia of the muscular wall fo the great vessels (arrow). (f). (i) In a severe case, formation of the endocardial cushion (asterisk) is markedly impaired and the AV valve is not formed. A large VSD is indicated by double arrows. Av, arterioventricular valve; pv, pulmonary vein.

Copyright

This image is from Yanagisawa H, J Clin Invest 2000 May;105(10):1373-82 and is displayed with the permission of the American Society for Clinical Investigation who owns the Copyright. J:62261

Associated Alleles

Symbol	Name
Ece1^tm1Reh	endothelin converting enzyme 1; targeted mutation 1, Robert E Hammer
Ece2^tm1Ywa	endothelin converting enzyme 2; targeted mutation 1, Masashi Yanagisawa

Associated Genotypes

Allelic Composition	Genetic Background
Ece1^tm1Reh/Ece1^tm1Reh Ece2^tm1Ywa/Ece2^tm1Ywa	involves: 129S6/SvEvTac * C57BL/6J
Ece1^tm1Reh/Ece1^tm1Reh	involves: 129S6/SvEvTac * C57BL/6J