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. Author manuscript; available in PMC: 2022 Apr 1.
Published in final edited form as: Cold Spring Harb Perspect Biol. 2021 Apr 1;13(4):a036764. doi: 10.1101/cshperspect.a036764

Figure 3. Murine models for conotruncal and aortic arch artery defects.

Figure 3.

(A) Diagram illustrates TOF, characterized by right ventricular hypertrophy, VSD (arrow), stenosis of pulmonary valve and sub-valvar area (*) in human heart. Comparable TOF phenotype was observed in mouse model of Crkol−/− at embryonic day 16.5 shown in B and C. (D, F) IAA (*) detected in human patients were recapitulated in Tbx1Neo2/Neo2 mouse model at E18.5 compared to wildtype mouse (E). (G, I) Representative diagram of a diseased heart display DORV and TA with VSD (arrow head) in human (G, I). Similar phenotypes were observed in Tbx1Neo2/Neo2 mice (H, J) at E18.5. RA: right atrium; RV: right ventricle; LA: left atrium; LV: left ventricle; AV: aortic valve; Ao/A: Aorta; Pa/P: Pulmonary artery; TOF: tetralogy of Fallot; IAA: Interrupted aortic arch; DORV: double outlet right ventricle; TA: truncus arteriosus; VSD: ventricular septal defect. (E, F, H, J) have been adapted from Guris et al. 2001 and Zhang and Baldini 2008 with permission.