In addition, transcriptomic and metabolomic analyses of the aorta in patients with Marfan syndrome (MFS, the major complication of which is the risk of ATAA) and mouse models reveal that the decreased expression of mitochondrial transcription factor A (TFAM) and mtDNA content in the aorta of patients and model groups repress the expression of mitochondrial oxidative respiration complex and mitochondrial function related proteins, instead, enhance the glycolytic related proteins expression, and eventually decrease the cellular oxygen consumption rate and increase the production of lactate. Here, TFAM is linked to Marfan syndrome.