Nature Communications (May 2021)

Aortic disease in Marfan syndrome is caused by overactivation of sGC-PRKG signaling by NO

  • Andrea de la Fuente-Alonso,
  • Marta Toral,
  • Alvaro Alfayate,
  • María Jesús Ruiz-Rodríguez,
  • Elena Bonzón-Kulichenko,
  • Gisela Teixido-Tura,
  • Sara Martínez-Martínez,
  • María José Méndez-Olivares,
  • Dolores López-Maderuelo,
  • Ileana González-Valdés,
  • Eusebio Garcia-Izquierdo,
  • Susana Mingo,
  • Carlos E. Martín,
  • Laura Muiño-Mosquera,
  • Julie De Backer,
  • J. Francisco Nistal,
  • Alberto Forteza,
  • Arturo Evangelista,
  • Jesús Vázquez,
  • Miguel R. Campanero,
  • Juan Miguel Redondo

DOI
https://doi.org/10.1038/s41467-021-22933-3
Journal volume & issue
Vol. 12, no. 1
pp. 1 – 18

Abstract

Read online

Aortic aneurysm and dissection, the major problem linked to Marfan syndrome (MFS), lacks effective pharmacological treatment. Here, the authors show that the NO pathway is overactivated in MFS and that inhibition of guanylate cyclase and cGMP-dependent protein kinase reverts MFS aortopathy in mice.