Paradoxical aortic stiffening and subsequent cardiac dysfunction in Hutchinson–Gilford progeria syndrome
Murtada SI, Kawamura Y, Caulk AW, Ahmadzadeh H, Mikush N, Zimmerman K, Kavanagh D, Weiss D, Latorre M, Zhuang ZW, Shadel GS, Braddock DT, Humphrey JD. Paradoxical aortic stiffening and subsequent cardiac dysfunction in Hutchinson–Gilford progeria syndrome. Journal Of The Royal Society Interface 2020, 17: 20200066. PMID: 32453981, PMCID: PMC7276555, DOI: 10.1098/rsif.2020.0066.Peer-Reviewed Original ResearchConceptsHutchinson-Gilford progeria syndromeSubsequent cardiac dysfunctionSmooth muscle functionPulse wave velocityUltra-rare disordersCardiovascular eventsDevastating sequelaeDiastolic dysfunctionSystolic functionCardiac dysfunctionProgeria syndromeVascular functionAortic functionMuscle functionEarly deathMouse modelTherapeutic windowCardiovascular dataBiomechanical phenotypingDysfunctionSyndromeExcessive accumulationBiomechanical functionArterial developmentPressure-related effects