#Progerin

#HGPS #Aortopathy

The age-related accumulation of immature Lamin A, #Progerin or Farnesyl-Prelamin A, ⏫Stretch-induced loss of aortic #SmoothMuscleCell

Comparative analysis among
1⃣LmnaG609G/+
2⃣Zmpste24-/-
3⃣SM22a-Cre::Zmpste f/f::LmnaPLAO/PLAO🐭

#CellDeathDis 2025
www.nature.com/articles/s41...

Lack of adverse vascular phenotypes in mice with postnatal induction of #EndothelialCell #Progerin
Lmna LCS/LCS::Cdh5-CreERT2
Tamoxifen 1.5-mo
Monitored till 22-mo

Normal lifespan
No #Progeria
No Cardiac Fibrosis
No aggravated Atherosclerosis

#AgingCell 2024
onlinelibrary.wiley.com/doi/10.1111/...

#HutchinsonGilfordProgeriaSyndrome

#Senescence/#Aging features of Isogenic HGPS #hiPSC-#EndothelialCell vs #SmoothMuscleCell

#Progerin <
Nuclear deformation <
#Senescence >
#DNADamage ~
#TelomereLength <
#Shelterin
#SASP
#Heterochromatin loss ~

#EHJOpen 2022
academic.oup.com/ehjopen/arti...