Oxidized mtDNA released into cytosol binds and activates the NLPR3 inflammasome directly in macrophage [67, 71]; activation of macrophage inflammasome is involved in hyperhomocysteinemia-aggravated AAA formation, as observed in vitro and in AngII-infused apolipoprotein E-deficient mice [2]. Here, APOE is linked to triple-A syndrome.