Induction of cardiac fibulin-4 protects against pressure overload-induced cardiac hypertrophy and heart failure

Abstract The prevailing view of fibulin-4 deficient mice is that the cardiac phenotype is the result of aortic and/or valvular disease. In the present study, we have tested whether the cardiac phenotype is, at least in part, the consequence of primary cardiac effects of fibulin-4. We have found fibu...

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Main Authors: E. D. van Deel, M. Snelders, N. van Vliet, L. te Riet, T. P. P. van den Bosch, L. R. Fiedler, A. C. C. van Spreeuwel, N. A. M. Bax, N. Boontje, C. M. Halabi, T. Sasaki, D. P. Reinhardt, J. van der Velden, C.V.C. Bouten, J. H. von der Thüsen, A. H. J. Danser, D. J. Duncker, M. D. Schneider, I. van der Pluijm, J. Essers
Format: Article
Language:English
Published: Nature Portfolio 2025-04-01
Series:Communications Biology
Online Access:https://doi.org/10.1038/s42003-025-08087-8
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Summary:Abstract The prevailing view of fibulin-4 deficient mice is that the cardiac phenotype is the result of aortic and/or valvular disease. In the present study, we have tested whether the cardiac phenotype is, at least in part, the consequence of primary cardiac effects of fibulin-4. We have found fibulin-4 expression to be activated throughout the myocardium in wildtype (fibulin-4+/+) C57Bl/6J;129 Sv mice subjected to transverse aortic constriction (TAC). In contrast, haploinsufficient fibulin-4+/R mice exposed to severe TAC do not show this increase in myocardial fibulin-4 expression, but display altered physical properties of myocardial tissue. Moreover, TAC-induced cardiac fibrosis, pulmonary congestion, and mortality are aggravated in fibulin-4+/R mice. In vitro investigations of myocardial tissue show that fibulin-4 deficiency results in cardiomyocyte hypertrophy, and a decreased beating frequency and contractile force. In conclusion, we demonstrate functions for fibulin-4 in cardiac homeostasis and show that reduced fibulin-4 expression drives myocardial disease in response to cardiac pressure overload, independent of aortic valvular pathology.
ISSN:2399-3642