Common and Distinctive Pathogenetic Features of Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia 1 and Hereditary Hemorrhagic Telangiectasia 2 Animal Models-Brief Report
DC Field | Value | Language |
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dc.contributor.author | Garrido-Martin, Eva M. | - |
dc.contributor.author | Nguyen, Ha-Long | - |
dc.contributor.author | Cunningham, Tyler A. | - |
dc.contributor.author | Choe, Se-woon | - |
dc.contributor.author | Jiang, Zhihua | - |
dc.contributor.author | Arthur, Helen M. | - |
dc.contributor.author | Lee, Young-Jae | - |
dc.contributor.author | Oh, S. Paul | - |
dc.date.available | 2020-02-28T16:41:45Z | - |
dc.date.created | 2020-02-06 | - |
dc.date.issued | 2014-10 | - |
dc.identifier.issn | 1079-5642 | - |
dc.identifier.uri | https://scholarworks.bwise.kr/gachon/handle/2020.sw.gachon/12238 | - |
dc.description.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. | - |
dc.language | 영어 | - |
dc.language.iso | en | - |
dc.publisher | LIPPINCOTT WILLIAMS & WILKINS | - |
dc.relation.isPartOf | ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY | - |
dc.subject | MOUSE | - |
dc.subject | ALK1 | - |
dc.title | Common and Distinctive Pathogenetic Features of Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia 1 and Hereditary Hemorrhagic Telangiectasia 2 Animal Models-Brief Report | - |
dc.type | Article | - |
dc.type.rims | ART | - |
dc.description.journalClass | 1 | - |
dc.identifier.wosid | 000344056100012 | - |
dc.identifier.doi | 10.1161/ATVBAHA.114.303984 | - |
dc.identifier.bibliographicCitation | ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY, v.34, no.10, pp.2232 - + | - |
dc.identifier.scopusid | 2-s2.0-84914171026 | - |
dc.citation.endPage | + | - |
dc.citation.startPage | 2232 | - |
dc.citation.title | ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY | - |
dc.citation.volume | 34 | - |
dc.citation.number | 10 | - |
dc.contributor.affiliatedAuthor | Lee, Young-Jae | - |
dc.contributor.affiliatedAuthor | Oh, S. Paul | - |
dc.type.docType | Article | - |
dc.subject.keywordAuthor | Alk1 protein, mouse | - |
dc.subject.keywordAuthor | arteriovenous malformations | - |
dc.subject.keywordAuthor | endoglin protein, mouse | - |
dc.subject.keywordAuthor | endothelial cells | - |
dc.subject.keywordAuthor | myocytes, smooth muscle | - |
dc.subject.keywordAuthor | telangiectasia, hereditary hemorrhagic | - |
dc.subject.keywordPlus | MOUSE | - |
dc.subject.keywordPlus | ALK1 | - |
dc.relation.journalResearchArea | Hematology | - |
dc.relation.journalResearchArea | Cardiovascular System & Cardiology | - |
dc.relation.journalWebOfScienceCategory | Hematology | - |
dc.relation.journalWebOfScienceCategory | Peripheral Vascular Disease | - |
dc.description.journalRegisteredClass | scie | - |
dc.description.journalRegisteredClass | scopus | - |
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