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

doi:10.1038/s41467-021-22933-3

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Article

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

Nature communications - 11 May 2021

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

Abstract excerpt

Thoracic aortic aneurysm, as occurs in Marfan syndrome, is generally asymptomatic until dissection or rupture, requiring surgical intervention as the only available treatment. Here, we show that nitric oxide (NO) signaling dysregulates actin cytoskeleton dynamics in Marfan Syndrome smooth muscle cells and that NO-donors induce Marfan-like aortopathy in wild-type mice, indicating that a marked increase in NO...

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Identifiers

PMID: 33976159
DOI: 10.1038/s41467-021-22933-3
PMCID: PMC8113458

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