Fibulin-4 deficiency increases TGF-β signalling in aortic smooth muscle cells due to elevated TGF-β2 levels

@inproceedings{Ramnath2015Fibulin4DI,
  title={Fibulin-4 deficiency increases TGF-β signalling in aortic smooth muscle cells due to elevated TGF-β2 levels},
  author={Natasja W. M. Ramnath and Lukas J. A. C. Hawinkels and Paula M. van Heijningen and Luuk te Riet and Madelon Paauwe and M. J. M. Vermeij and Alexander Hj Danser and Roland Kanaar and Peter Ten Dijke and Jeroen Essers},
  booktitle={Scientific reports},
  year={2015}
}
Fibulins are extracellular matrix proteins associated with elastic fibres. Homozygous Fibulin-4 mutations lead to life-threatening abnormalities such as aortic aneurysms. Aortic aneurysms in Fibulin-4 mutant mice were associated with upregulation of TGF-β signalling. How Fibulin-4 deficiency leads to deregulation of the TGF-β pathway is largely unknown. Isolated aortic smooth muscle cells (SMCs) from Fibulin-4 deficient mice showed reduced growth, which could be reversed by treatment with TGF… CONTINUE READING
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The authors declare no competing financial interests

  • Hawinkels, L. J. et al. Active TGF-β1 correlates with myofibroblasts, +14 authors J. E. supervised the project. All authors reviewed th interests
  • How to cite this article: Ramnath, N. W. M. et al…
  • 2015

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