#BicuspidAorticValve prevalence (6% vs 1~2% in general population) & related aortic outcomes in heritable #Aortopathy (TGFB 7 genes; ECM 7 genes; SMC contractile 5 genes; Other genes: HCN4 SLC2A10)
#MontalcinoAorticConsortium
N=816
#AortaEd
medRxiv 2025
www.medrxiv.org/content/10.1...
Zebrafishology, study design guidelines for rigorous & reproducible data using zebrafish
Actually, this is an entertaining reading piece for a non-fishman like me😀
Reminder: Zebrafish is an emerging model of #Aortopathy!
Victoria Bedell lab #CommunBiol 2025
www.nature.com/articles/s42...
#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...
Outstanding talk by @OHSUCardio Vascular Surgery Head Dr. Sherene Shalhub on examining surgical failures in #aortopathy to build a model of understanding vascular catastrophes - look for her upcoming publication! @OHSUSOM @OHSUNews @ThinkAorta @ThinkAortaUS @JohnRitterFdn
Evidence for an active role of #EndothalialCell in #Aortopathy
EC TRPV4 activity⏫
🐭#AbdominalAorticAneurysm (local elastase)
via⏫EC #Apoptosis (how does this propagate to #SmoothMuscleCell loss) w/o altering #NitricOxide
#AortaEd
#EurJPharmacol 2024
www.sciencedirect.com/science/arti...
#EndMT #Aortopathy #MarfanSyndrome
A cascade of TGFβ-miR-632-DNAJB6 (#LimbGirdleMuscularDystrophy 1E)-βCatenin in both #EndothelialCell & #SmoothMuscleCell
miR-632 mimics delivery (lipofectamine) to cultured human aortic fragments
#IJMS_MDPI 2023
www.mdpi.com/1422-0067/24...
#ClonalHematopoiesis #AorticAneurysm
#AortaEd
CH driver gene JAK2 V617F could be causal to #Aortopathy 😎
Mainly a role in Myeloid cells
See the intriguing Mo/Mφ population shift b/w dilated & aneurysmal LysM-Cre:JAK2 V617F WT/Flex aortas😁
#NatureComms 2022
www.nature.com/articles/s41...
Integrated transcriptomic analysis of🙍♂️🐭Marfan Aorta➡️
A pathogenic role of HIPK2, an upstream kinase of TGFβ/SMAD3, in Marfan #Aortopathy
Adult Hipk2 KO or BT173 (HIPK2 inhibitor) in mgR/mgR Marfan🐭rescues
Lifespan &
Aortic damage
#AortaEd
#ATVB 2021
www.ahajournals.org/doi/full/10....
Dilation of Aortic root & Ascending aorta during pregnancy in 38 ♀️+Genetic #Aortopathy
Aorta Not dilated at baseline
Mean Δaorticφ
(baseline->3rd trimester)
Annulus 2.0 mm⬆️
Sinus ~1.2 mm⬆️
Ascending ~1.9 mm⬆️
#AortaEd
#OBMedicine 2024
journals.sagepub.com/doi/10.1177/...
BAPN (LOX inhibitor) #Aortopathy in #Zebrafish🐟
Larvae 50 μM, 3 d➡️
28%⏫Aorticφ
75% Aortic arch malformation
Adult 1 mM, 22 d➡️
2.5-fold⏫Aortic CSA
3-fold⏫Aortic wall thickness
58% Hemorrhage (of which vascular beds?)
Elastin disruption
Anim Cells Syst 2024
www.tandfonline.com/doi/full/10....
#EndMT #Aortopathy #MarfanSyndrome
A cascade of TGFβ-miR-632-DNAJB6 (#LimbGirdleMuscularDystrophy 1E)-βCat in both #EndothelialCell & #SmoothMuscleCell
miR-632 mimics delivery (lipofectamine) to cultured human aortic fragments
#AortaEd
#IJMSMDPI 2023
www.mdpi.com/1422-0067/24...
