PMC:1635039 / 23292-24927 JSONTXT

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While Tgfbr2/Wnt1-Cre mutants as well as mice deficient in Tgf-β2 display the PTA type A4 (truncus arteriosus with interrupted aortic arch [30]), Alk5/Wnt1-Cre mutants reported here demonstrate earlier patterning defects of the PAAs, which is particularly obvious in the 3rd pair of the PAAs. Moreover, the Alk5/Wnt1-Cre mutants display an abnormal patterning of the aortic sac and defective AP septation leading to PTA, reminiscent of type A2 (= truncus artriosus with no main pulmonary artery segment present [30]). However, our results also demonstrate that significant hypoplasia of the aortic sac leads to a severe shortening of the ascending truncal arch, which masks possible defects in derivatives of the 4th PAAs, i.e., interruption of the aortic arch. 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