Trem-1 inhibitors for the treatment of marfan syndrome

Abstract

Marfan Syndrome is a pathology responsible for a high morbidity and mortality. Apart from surgery, treatment options are limited. It is therefore essential to develop new pharmacological approaches to limit aortic dilatation and/or rupture. Now, the inventors have demonstrated a critical role for TREM-1 in the pathophysiology of ascending aortopathy related to Marfan disease, In particular, the inventors showed that i) TREM-1 is expressed by macrophages in the aortic wall of Marfan patients, ii) TREM-1 is expressed by macrophages in the aortic wall of Marfan mice, and iii) TREM-1 deficiency protects against aortic rupture in a mouse model of Marfan syndrome. Blocking TREM-1 could therefore be a therapeutic approach for Marfan-related aortopathy.

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