{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T03:58:19Z","timestamp":1767153499825,"version":"build-2238731810"},"reference-count":53,"publisher":"Portland Press Ltd.","issue":"5","license":[{"start":{"date-parts":[[2019,9,19]],"date-time":"2019-09-19T00:00:00Z","timestamp":1568851200000},"content-version":"vor","delay-in-days":141,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2019,5,31]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Hereditary haemorrhagic telangiectasia (HHT) is a progressive vascular disease with high mortality and prevalence. There is no effective treatment of HHT due to the lack of comprehensive knowledge of its underlying pathological mechanisms. The majority of HHT1 patients carry endoglin (ENG) mutations. Here, we used Danio rerio (zebrafish) as an in vivo model to investigate the effects of endoglin knockdown on vascular development. According to phylogenetic analyses and amino acid sequence similarity analyses, we confirmed that endoglin is conserved in vertebrates and descended from a single common ancestor. Endoglin is highly expressed in the vasculature beginning at the segmentation period in zebrafish. Upon endoglin knockdown by morpholinos, we observed disruption in the intersegmental vessels (ISVs) and decreased expression of several vascular markers. RNA sequencing (RNA-Seq) results implied that the BMP-binding endothelial regulator (bmper) is a gene affected by endoglin knockdown. Rescue experiments demonstrated that overexpression of bmper significantly increased the number of endothelial cells (ECs) and reduced the defects at ISVs in zebrafish. Moreover, there was enhanced tube formation in ENG mutant ECs derived from a HHT patient after human recombinant BMPER (hrBMPER) stimulation. Taken together, our results suggest that bmper, a potential downstream gene of ENG, could be targeted to improve vascular integrity in HHT.<\/jats:p>","DOI":"10.1042\/bsr20182320","type":"journal-article","created":{"date-parts":[[2019,5,7]],"date-time":"2019-05-07T14:11:41Z","timestamp":1557238301000},"update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Endoglin is a conserved regulator of vasculogenesis in zebrafish \u2013 implications for hereditary haemorrhagic telangiectasia"],"prefix":"10.1042","volume":"39","author":[{"given":"Ding","family":"Zhang","sequence":"first","affiliation":[{"name":"Department of Cell Biology, State Key Laboratory of Medical Molecular Biology, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100005, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fang","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Cell Biology, State Key Laboratory of Medical Molecular Biology, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100005, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiuli","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences and School of Basic Medicine, Peking Union Medical College, Beijing 100005, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bao","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Vascular Surgery, Peking Union College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing 100005, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jingyu","family":"Chen","sequence":"additional","affiliation":[{"name":"Wuxi Lung Transplant Center, Wuxi People\u2019s Hospital affiliated to Nanjing Medical University, Wuxi 214023, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9715-8100","authenticated-orcid":false,"given":"Jun","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Cell Biology, State Key Laboratory of Medical Molecular Biology, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100005, China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"288","published-online":{"date-parts":[[2019,5,21]]},"reference":[{"key":"2021111619361393600_B1","doi-asserted-by":"publisher","first-page":"203","DOI":"10.1016\/j.blre.2010.07.001","article-title":"Hereditary haemorrhagic telangiectasia: pathophysiology, diagnosis and treatment","volume":"24","author":"Shovlin","year":"2010","journal-title":"Blood Rev."},{"key":"2021111619361393600_B2","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1097\/GIM.0b013e3182136d32","article-title":"Hereditary hemorrhagic telangiectasia: an overview of diagnosis, management, and pathogenesis","volume":"13","author":"McDonald","year":"2011","journal-title":"Genet. 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