#Aortopathy

Hao Yin @HaoYin@qoto.org

IL-11 as a therapeutic target for lung #Fibrosis #Emphysema in #MarfanSyndrome

In Marfan, IL11-GFP reporter is active in #EndothelialCell #SmoothMuscleCell Fibroblast but not alveolar epithelia

Also don't miss the story on IL-11 as mediator of Mafan #Aortopathy from Circ Res 2022

Hao Yin @HaoYin@qoto.org

Bi-allelic Emilin 1 LOF causes #Aortopathy

EMILIN1 deficiency->
Fibulin 4
LOX
#TGFβ signals
#Elastogenesis
#CollagenFibrillogenesis

Dr. Gerhard Sengle & Bert Callewaert labs Am J Hum Genet 2022
https://www.cell.com/ajhg/fulltext/S0002-9297(22)00458-X

Hao Yin @HaoYin@qoto.org

In #BicuspidAorticValve #Aortopathy, #Aorta-enriched plasma cell-free DNA methylation correlated with ascending aortic #Apoptosis in regions of elevated #WallShearStress (4D flow MRI), but Not aortic diameter

An interesting method to identify tissue-enriched DNA methylation in this paper

24 candidate aorta-enriched differentially methylated regions
4 used in this study

Hao Yin @HaoYin@qoto.org

10k steps/day physical activity (Garmin) for 6 mo Slow down enlargement rate of #AorticRoot (Echo) in #MarfanSyndrome

15 Ctrl 12 yr+24 Intervention 14 yr

0.13 vs -0.01 cm/yr

Can anyone rec papers on the normal growth rate of aorta dimension in healthy children/teenagers?

Hao Yin @HaoYin@qoto.org

Phospholipase Cε #Aortopathy

Plce1 KO are susceptible to ANGII-induced ascending #AorticAneurysm+Dissection

Role of adventitial myofibroblast cytokines?

Dr. Alan Smrcka, Santhi Ganesh, Markus Bitzer labs AJP Heart Circ 2022
https://journals.physiology.org/doi/abs/10.1152/ajpheart.00262.2022