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

Dr. Stuart A Cook & Wei Wen Lim lab ATVB 2023
https://www.ahajournals.org/doi/10.1161/ATVBAHA.122.318802

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

https://www.ahajournals.org/doi/10.1161/CIRCRESAHA.121.320381

Bi-allelic Emilin 1 LOF causes #Aortopathy

EMILIN1 deficiency👤🐭->
⏬Fibulin 4
⏬LOX
⏫#TGFβ signals
⏬#Elastogenesis
⏬#CollagenFibrillogenesis

Pediatric #AscendingAorticAneurysm

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

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

Roadmap Epigenomics
BluePrint Epigenome
Metilene
https://www.bioinf.uni-leipzig.de/Software/metilene/

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

Dr. Steven Greenway lab Clin Epigenetics 2021
https://clinicalepigeneticsjournal.biomedcentral.com/articles/10.1186/s13148-021-01137-y

#Excercise #Aortopathy

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 🤠

Dr. Elif S.S. Tierney lab JAHA 2022 @MarfanFdn
https://www.ahajournals.org/doi/10.1161/JAHA.122.027598

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

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