Multimodality imaging and transcriptomics to phenotype mitral valve dystrophy in a unique knock-in Filamin-A rat model.

The KI FlnA-P637Q rat model mimics human myxomatous mitral valve dystrophy, offering a unique opportunity to decipher pathophysiological mechanisms related to this disease. Extracellular matrix organization, epithelial cell migration, response to mechanical stress, and a central contribution of immune cells are highlighted as the main signaling pathways leading to myxomatous mitral valve dystrophy. Our findings pave the road to decipher underlying molecular mechanisms and the specific role of distinct cell populations in this context.