Common and Distinctive Pathogenetic Features of Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia 1 and Hereditary Hemorrhagic Telangiectasia 2 Animal Models-Brief Report
- Authors
- Garrido-Martin, Eva M.; Nguyen, Ha-Long; Cunningham, Tyler A.; Choe, Se-woon; Jiang, Zhihua; Arthur, Helen M.; Lee, Young-Jae; Oh, S. Paul
- Issue Date
- Oct-2014
- Publisher
- LIPPINCOTT WILLIAMS & WILKINS
- Keywords
- Alk1 protein, mouse; arteriovenous malformations; endoglin protein, mouse; endothelial cells; myocytes, smooth muscle; telangiectasia, hereditary hemorrhagic
- Citation
- ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY, v.34, no.10, pp.2232 - +
- Journal Title
- ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY
- Volume
- 34
- Number
- 10
- Start Page
- 2232
- End Page
- +
- URI
- https://scholarworks.bwise.kr/gachon/handle/2020.sw.gachon/12238
- DOI
- 10.1161/ATVBAHA.114.303984
- ISSN
- 1079-5642
- Abstract
- Objective-Hereditary hemorrhagic telangiectasia is a genetic disorder characterized by visceral and mucocutaneous arteriovenous malformations (AVMs). Clinically indistinguishable hereditary hemorrhagic telangiectasia 1 and hereditary hemorrhagic telangiectasia 2 are caused by mutations in ENG and ALK1, respectively. In this study, we have compared the development of visceral and mucocutaneous AVMs in adult stages between Eng- and Alk1-inducible knockout (iKO) models. Approach and Results-Eng or Alk1 were deleted from either vascular endothelial cells (ECs) or smooth muscle cells in adult stages using Scl-CreER and Myh11-CreER lines, respectively. Latex perfusion and intravital spectral imaging in a dorsal skinfold window chamber system were used to visualize remodeling vasculature during AVM formation. Global Eng deletion resulted in lethality with visceral AVMs and wound-induced skin AVMs. Deletion of Alk1 or Eng in ECs, but not in smooth muscle cells, resulted in wound-induced skin AVMs. Visceral AVMs were observed in EC-specific Alk1-iKO but not in Eng-iKO. Intravital spectral imaging revealed that Eng-iKO model exhibited more dynamic processes for AVM development when compared with Alk1-iKO model. Conclusions-Both Alk1- and Eng-deficient models require a secondary insult, such as wounding, and ECs are the primary cell type responsible for the pathogenesis. However, Alk1 but not Eng deletion in ECs results in visceral AVMs.
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