WWP2 regulates pathological cardiac fibrosis by modulating SMAD2 signaling
File(s)WWP2RegulatesPathologicalCardiacFibrosis.pdf (4.2 MB)
Published version
Author(s)
Type
Journal Article
Abstract
Cardiac fibrosis is a final common pathology in inherited and acquired heart diseases that causes cardiac electrical and pump failure. Here, we use systems genetics to identify a pro-fibrotic gene network in the diseased heart and show that this network is regulated by the E3 ubiquitin ligase WWP2, specifically by the WWP2-N terminal isoform. Importantly, the WWP2-regulated pro-fibrotic gene network is conserved across different cardiac diseases characterized by fibrosis: human and murine dilated cardiomyopathy and repaired tetralogy of Fallot. Transgenic mice lacking the N-terminal region of the WWP2 protein show improved cardiac function and reduced myocardial fibrosis in response to pressure overload or myocardial infarction. In primary cardiac fibroblasts, WWP2 positively regulates the expression of pro-fibrotic markers and extracellular matrix genes. TGFβ1 stimulation promotes nuclear translocation of the WWP2 isoforms containing the N-terminal region and their interaction with SMAD2. WWP2 mediates the TGFβ1-induced nucleocytoplasmic shuttling and transcriptional activity of SMAD2.
Date Issued
2019-08-09
Date Acceptance
2019-07-19
Citation
Nature Communications, 2019, 10 (1), pp.1-19
ISSN
2041-1723
Publisher
Nature Research
Start Page
1
End Page
19
Journal / Book Title
Nature Communications
Volume
10
Issue
1
Copyright Statement
© The Author(s) 2019. Open Access. This article is licensed under a Creative Commons
Attribution 4.0 International License, which permits use, sharing,
adaptation, distribution and reproduction in any medium or format, as long as you give
appropriate credit to the original author(s) and the source, provide a link to the Creative
Commons license, and indicate if changes were made. The images or other third party
material in this article are included in the article’s Creative Commons license, unless
indicated otherwise in a credit line to the material. If material is not included in the
article’s Creative Commons license and your intended use is not permitted by statutory
regulation or exceeds the permitted use, you will need to obtain permission directly from
the copyright holder. To view a copy of this license, visit http://creativecommons.org/
licenses/by/4.0/.
Attribution 4.0 International License, which permits use, sharing,
adaptation, distribution and reproduction in any medium or format, as long as you give
appropriate credit to the original author(s) and the source, provide a link to the Creative
Commons license, and indicate if changes were made. The images or other third party
material in this article are included in the article’s Creative Commons license, unless
indicated otherwise in a credit line to the material. If material is not included in the
article’s Creative Commons license and your intended use is not permitted by statutory
regulation or exceeds the permitted use, you will need to obtain permission directly from
the copyright holder. To view a copy of this license, visit http://creativecommons.org/
licenses/by/4.0/.
Sponsor
Leducq Foundation for Cardiovascular Research
Commission of the European Communities
Heart Research UK
Fondation Leducq
Fondation Leducq
British Heart Foundation
Medical Research Council (MRC)
British Heart Foundation
British Heart Foundation
Identifier
https://www.nature.com/articles/s41467-019-11551-9
Grant Number
16CVD03
289600
RG2657/17/19
11 CVD-01
11 CVD-01
RM/13/1/30157
MR/M003191/1
SP/10/10/28431
FS/11/25/28740
Subjects
Science & Technology
Multidisciplinary Sciences
Science & Technology - Other Topics
E3 UBIQUITIN LIGASE
TGF-BETA
GENE-EXPRESSION
HEART-FAILURE
BLOOD-PRESSURE
FIBROBLAST
REVEALS
RAT
IDENTIFICATION
DETERMINANT
Adolescent
Adult
Aged
Animals
Cardiomyopathies
Extracellular Matrix Proteins
Female
Fibrosis
Gene Expression Regulation
Gene Regulatory Networks
Genetic Predisposition to Disease
Heart Diseases
Humans
Male
Mice
Mice, Transgenic
Middle Aged
Protein Isoforms
Smad2 Protein
Transforming Growth Factor beta
Ubiquitin-Protein Ligases
Young Adult
Animals
Mice, Transgenic
Humans
Mice
Heart Diseases
Cardiomyopathies
Genetic Predisposition to Disease
Fibrosis
Ubiquitin-Protein Ligases
Transforming Growth Factor beta
Protein Isoforms
Extracellular Matrix Proteins
Gene Expression Regulation
Adolescent
Adult
Aged
Middle Aged
Female
Male
Smad2 Protein
Gene Regulatory Networks
Young Adult
Publication Status
Published
Article Number
3616
Date Publish Online
2019-08-09