To gain mechanistic insights into the effects of the absence of LH1 on the formation of dissecting AAA, we performed whole-transcriptomic analysis, which demonstrated that LH1 deficiency aggravated gene transcription alterations; in particular, Thbs1 expression was markedly upregulated in the aortas of Ang II-treated Plod1-/-mice. This evidence concerns the gene PLOD1 and triple-A syndrome.