Posttranslational modifications in Marfan’s disease mediated by myeloperoxidase (A04+)

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This project aims to uncover the role of the innate immune system in Marfan Syndrome (MFS) and will define the interplay between extracellular matrix proteins, matrix-degrading enzymes, and myeloperoxidase (MPO) in MFS-related aortic disease. We hypothesize that innate immune cells extravasating into the aorta contribute to disease progression by their capacity to produce MPO, which exerts potent matrix destabilizing effects and affects the structural integrity of the vessel. Once the causality has been established, we propose to pharmacologically inhibit MPO in murine models of MFS as a new therapeutic strategy against aortic disease.