Smooth muscle cell proliferation in fetal elastin heterozygous versus control mice, suggests mechanism underlying Williams Syndrome cardiac disease

Authors

• A Watson
• M Levin
• Z Yu
• B Kozel

Abstract

Williams syndrome is caused by the deletion of 26-28 genes, including ELN on chromosome 7q11.23. Elastin, an extracellular matrix protein, is the major component of elastic fibers and is responsible for the elastic and rheologic properties of arteries. Normal deposition of elastin is a critical event in vessel development. Using the Eln+/- mouse we investigated the effects of elastin deficiency on embryonic development. Though many cardiovascular diseases have been characterized by defective lamellae and excess smooth muscle cells (SMC), the mechanism linking these pathological features is currently unknown. The aim of our study was to assess the timing of smooth muscle and endothelial proliferation in the carotid arteries in Eln+/- and WT mice. The BrdU alternative, Click-iT EdU, was injected at multiple time points throughout embryonic development to mark proliferating cells in WT and Eln+/- mice. Preliminary data show increased percentage of click-it positive smooth muscle cells marked in the Eln+/- coronaries, during the earlier embryonic time points in comparison to the WT. By contrast, no differences were seen in endothelial cells between Eln+/- and WT.

Scientific Focus Area: Developmental Biology

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