Subsequently, mechanistic studies revealed that NR1D1 deficiency restored the dysregulation and mitochondrial dysfunction of its direct transcriptional target ACO2 during early Ang II infusion prior to AAA formation, and that supplementation with α-ketoglutarate, a downstream ACO2 metabolite, prevented and treated AAA in mice in an NR1D1-dependent manner within VSMCs [107]. This evidence concerns the gene AGT and triple-A syndrome.