Lineage-specific events underlie aortic root aneurysm pathogenesis in Loeys-Dietz syndrome

Volume: 129, Issue: 2, Pages: 659 - 675
Published: Feb 5, 2019
Abstract
The aortic root is the predominant site for development of aneurysm caused by heterozygous loss-of-function mutations in positive effectors of the transforming growth factor-β (TGF-β) pathway. Using a mouse model of Loeys-Dietz syndrome (LDS) that carries a heterozygous kinase-inactivating mutation in TGF-β receptor I, we found that the effects of this mutation depend on the lineage of origin of vascular smooth muscle cells (VSMCs). Secondary...
Paper Details
Title
Lineage-specific events underlie aortic root aneurysm pathogenesis in Loeys-Dietz syndrome
Published Date
Feb 5, 2019
Volume
129
Issue
2
Pages
659 - 675
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