The instant invention provides methods and compositions for the treatment and prevention of Marfan syndrome and related diseases, disorders and conditions. The invention further provides pharmaceutical compositions and kits for the treatment and prevention of Marfan syndrome and related diseases, disorders and conditions.Cette invention concerne des méthodes et des compositions permettant de traiter et de prévenir le syndrome de Marfan et les maladies, les troubles et les affections associés. L'invention concerne par ailleurs des compositions pharmaceutiques et des kits associés.