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Arterial disease

Sphingolipid signaling links elastin deficiency to arterial hyper-muscularization and congenital heart disease

Defects in elastin trigger hyperproliferation of smooth muscle cells, which leads to arterial and congenital heart diseases. Research now shows that elastin deficiency induces SPHK1 and S1P signaling by EGR1 in SMCs, and inhibitors of SPHK1 or S1PR1 attenuate smooth muscle cell proliferation and mitigate aortic disease.

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Fig. 1: Defects in elastin and elastic fibers lead to SMC hyperproliferation, resulting in SVAS.

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Correspondence to Besim Ogretmen.

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Darawshi, O., Ogretmen, B. Sphingolipid signaling links elastin deficiency to arterial hyper-muscularization and congenital heart disease. Nat Cardiovasc Res 5, 10–11 (2026). https://doi.org/10.1038/s44161-025-00763-6

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