Lineage-specific events underlie aortic root aneurysm pathogenesis in Loeys-Dietz syndrome

Journal
Journal of Clinical Investigation
ISSN
0021-9738
1558-8238
Date Issued
2019
Author(s)
Elena Gallo MacFarlane
Sarah J. Parker
Joseph Y. Shin
Shira G. Ziegler
Tyler J. Creamer
Rustam Bagirzadeh
Djahida Bedja
Yichun Chen
Katherine Weissler
Pamela A. Frischmeyer-Guerrerio
Mark E. Lindsay
Jennifer P. Habashi
Harry C. Dietz
Type
Resource Types::text::journal::journal article
Scopus ID
2-s2.0-85060877038
WoS ID
WOS:000457479300027
DOI
URL
URL Institutional Repository
Cite this document
MacFarlane, E. G., Parker, S. J., Shin, J. Y., Ziegler, S. G., Creamer, T. J., Bagirzadeh, R., Bedja, D., Chen, Y., Calderon, J. F., Weissler, K., Frischmeyer-Guerrerio, P. A., Lindsay, M. E., Habashi, J. P., & Dietz, H. C. (2019). Lineage-specific events underlie aortic root aneurysm pathogenesis in Loeys-Dietz syndrome. Journal of Clinical Investigation, 129(2), 659-675. https://doi.org/10.1172/JCI123547
Project(s)
Subjects
smooth-muscle-cells

; 

growth-factor-beta

; 

of-function mutations

; 

tgf-beta

; 

angiotensin-ii

; 

embryological-origin

; 

latent tgf-beta-1

; 

mouse model

; 

marfan

; 

receptor

; 

animals

; 

disease models, animal

; 

humans

; 

loeys-dietz syndrome

; 

mice

; 

mice, mutant strains

; 

muscle, smooth, vascular

; 

myocytes, smooth muscle

; 

receptor, angiotensin, type 1

; 

signal transduction

; 

smad2 protein

; 

smad3 protein

; 

angiotensin 1 receptor

; 

smad2 protein

; 

smad3 protein

; 

transforming growth factor beta

; 

agtr1a protein, mouse

; 

angiotensin 1 receptor

; 

smad2 protein

; 

smad2 protein, mouse

; 

smad3 protein

; 

smad3 protein, mouse

; 

animal cell

; 

animal experiment

; 

animal model

; 

animal tissue

; 

aortic aneurysm

; 

aortic root

; 

aortic wall

; 

article

; 

cell lineage

; 

controlled study

; 

gene deletion

; 

in vitro study

; 

in vivo study

; 

loeys dietz syndrome

; 

loss of function mutation

; 

mouse

; 

neural crest

; 

nonhuman

; 

pathogenesis

; 

priority journal

; 

protein expression

; 

protein phosphorylation

; 

tgf beta signaling

; 

vascular smooth muscle cell

; 

animal

; 

disease model

; 

embryology

; 

genetics

; 

human

; 

loeys dietz syndrome

; 

metabolism

; 

mutant mouse strain

; 

pathology

; 

signal transduction

; 

smooth muscle cell

; 

vascular smooth muscle