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hiPSC Modeling of Lineage-Specific Smooth Muscle Cell Defects Caused by TGFBR1 A230T Variant, and Its Therapeutic Implications for Loeys-Dietz Syndrome

4 Aug 2021

Abstract excerpt

Background: Loeys-Dietz syndrome (LDS) is an inherited disorder predisposing individuals to thoracic aortic aneurysm and dissection. Currently, there are no medical treatments except surgical resection. Although the genetic basis of LDS is well-understood, molecular mechanisms underlying the disease remain elusive, impeding the development of a therapeutic strategy. In addition, aortic smooth muscle cells (SMCs)...

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PMID: 34346740
DOI: 10.1161/circulationaha.121.054744

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