The present study uses tissue-specific inducible CCN2-deficient mice (CCN2fl/fl–Myh11–Cre-ERT2) and murine models of AAA (induced by systemic angiotensin II [Ang II] infusion or local adventitia elastase exposure) to characterize the role of SMC-specific CCN2 in the development of AAA. This evidence concerns the gene MAPK3 and triple-A syndrome.