Document Type
Article
Publication Date
2019
Keywords
JGM
JAX Source
J Cell Mol Med 2019; 23:2526-2535
ISSN
1582-4934
PMID
30677223
DOI
https://doi.org/10.1111/jcmm.14137
Abstract
Marfan syndrome (MFS) is an autosomal dominant genetic disorder caused by mutations in the FBN1 gene. Although many peripheral tissues are affected, aortic complications, such as dilation, dissection and rupture, are the leading causes of MFS-related mortality. Aberrant TGF-beta signalling plays a major role in the pathophysiology of MFS. However, the contributing mechanisms are still poorly understood. Here, we aimed at identifying novel aorta-specific pathways involved in the pathophysiology of MFS. For this purpose, we employed the Fbn1 under-expressing mgR/mgR mouse model of MFS. We performed RNA-sequencing of aortic tissues of 9-week-old mgR/mgR mice compared with wild-type (WT) mice. With a false discovery rate
Recommended Citation
Bhushan R,
Altinbas L,
Jäger M,
Zaradzki M,
Lehmann D,
Timmermann B,
Clayton N,
Zhu Y,
Kallenbach K,
Kararigas G,
Robinson P.
An integrative systems approach identifies novel candidates in Marfan syndrome-related pathophysiology. J Cell Mol Med 2019; 23:2526-2535
Comments
Open access under the terms of the Creative Commons Attribution License