Arterial-Lymphatic-Like Endothelial Cells Appear in Hereditary Hemorrhagic Telangiectasia 2 and Contribute to Vascular Leakage and Arteriovenous Malformations

10.1161/CIRCULATIONAHA.124.070925

Affiliations
Yang Yang, Xiuju Wu, Yan Zhao, Daoqin Zhang, Li Zhang, Xinjiang Cai, Jaden Ji, Zheng Jing, Kristina I Boström, Yucheng Yao

DOI: 10.1161/CIRCULATIONAHA.124.070925

Abstract
BACKGROUND: Arteriovenous malformations (AVMs) are characteristic of hereditary hemorrhagic telangiectasia. Loss-of-function mutations in the activin receptor-like kinase 1 ( METHODS: Endothelial-specific deletion of RESULTS: We performed endothelial-specific deletion of CONCLUSIONS: Together, our studies revealed the mechanistic underpinnings of ALK1 signaling in regulating the endothelial phenotype and provided possibilities for new therapeutic strategies in hemorrhagic telangiectasia type 2.

Keywords
arterial endothelial cells, arteriovenous malformations, hereditary hemorrhagic telangiectasia 2

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Arterial-Lymphatic-Like Endothelial Cells Appear in Hereditary Hemorrhagic Telangiectasia 2 and Contribute to Vascular Leakage and Arteriovenous Malformations

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