NOTCH3 as a modulator of vascular disease: a target in elastin deficiency and arterial pathologies.

NOTCH3 as a modulator of vascular disease: a target in elastin deficiency and arterial pathologies.
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NOTCH3 作为血管疾病的调节剂:弹性蛋白缺乏和动脉病理的靶点。

DOI:
10.1172/jci157007
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发表时间:
2022-03-01
期刊:
The Journal of clinical investigation
影响因子:
--
通讯作者:
Liaw L
Liaw L
中科院分区:
其他
文献类型:
--
作者:
Malka K;Liaw L

文献摘要

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During blood vessel disease, vascular smooth muscle cell (VSMC) expansion and interaction with the matrix trigger changes in gene expression and phenotype. In this issue of the JCI, Dave et al. discover a signaling network that drives VSMC expansion and vascular obstruction caused by elastin insufficiency. Using a combination of gene-targeted mice, tissues and cells from patients with Williams-Beuren syndrome, and targeting of elastin in human VSMCs, the authors identified VSMC-derived NOTCH3 signaling as a critical mediator of aortic hypermuscularization and loss of vascular patency. NOTCH3-specific therapies or therapies that target downstream molecular pathways may provide opportunities to minimize VSMC growth and treat cardiovascular disease with minimal side effects.