Discovering and targeting mitochondrial loss in NOTCH1-related aortic aneurysm

Abstract

We established a mouse model of progressive aortic aneurysm induced by conditional Notch1 deficiency in vascular smooth muscle cells. Notch1 deficiency impairs the transcription of genes involved in mitochondrial fusion and biogenesis, thereby triggering aortic pathology. Early intervention to enhance these mitochondrial processes could potentially slow disease progression.