{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T12:04:44Z","timestamp":1790424284265,"version":"4.1.0"},"reference-count":66,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"7","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["ATVB"],"published-print":{"date-parts":[[2018,7]]},"abstract":"<jats:sec>\n            <jats:title>Objective\u2014<\/jats:title>\n            <jats:p>Endothelial cells store VWF (von Willebrand factor) in rod-shaped secretory organelles, called Weibel-Palade bodies (WPBs). WPB exocytosis is coordinated by a complex network of Rab GTPases, Rab effectors, and SNARE (soluble NSF attachment protein receptor) proteins. We have previously identified STXBP1 as the link between the Rab27A-Slp4-a complex on WPBs and the SNARE proteins syntaxin-2 and -3. In this study, we investigate the function of syntaxin-3 in VWF secretion.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Approach and Results\u2014<\/jats:title>\n            <jats:p>\n              In human umbilical vein endothelial cells and in blood outgrowth endothelial cells (BOECs) from healthy controls, endogenous syntaxin-3 immunolocalized to WPBs. A detailed analysis of BOECs isolated from a patient with variant microvillus inclusion disease, carrying a homozygous mutation in\n              <jats:italic>STX3<\/jats:italic>\n              (STX3\n              <jats:sup>\u2212\/\u2212<\/jats:sup>\n              ), showed a loss of syntaxin-3 protein and absence of WPB-associated syntaxin-3 immunoreactivity. Ultrastructural analysis revealed no detectable differences in morphology or prevalence of immature or mature WPBs in control versus STX3\n              <jats:sup>\u2212\/\u2212<\/jats:sup>\n              BOECs. VWF multimer analysis showed normal patterns in plasma of the microvillus inclusion disease patient, and media from STX3\n              <jats:sup>\u2212\/\u2212<\/jats:sup>\n              BOECs, together indicating WPB formation and maturation are unaffected by absence of syntaxin-3. However, a defect in basal as well as Ca\n              <jats:sup>2+<\/jats:sup>\n              - and cAMP-mediated VWF secretion was found in the STX3\n              <jats:sup>\u2212\/\u2212<\/jats:sup>\n              BOECs. We also show that syntaxin-3 interacts with the WPB-associated SNARE protein VAMP8 (vesicle-associated membrane protein-8).\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions\u2014<\/jats:title>\n            <jats:p>Our data reveal syntaxin-3 as a novel WPB-associated SNARE protein that controls WPB exocytosis.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/atvbaha.117.310701","type":"journal-article","created":{"date-parts":[[2018,6,7]],"date-time":"2018-06-07T09:00:10Z","timestamp":1528362010000},"page":"1549-1561","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":44,"title":["Weibel-Palade Body Localized Syntaxin-3 Modulates Von Willebrand Factor Secretion From Endothelial Cells"],"prefix":"10.1161","volume":"38","author":[{"given":"Maaike","family":"Schillemans","sequence":"first","affiliation":[{"name":"From the Plasma Proteins, Sanquin Research and Landsteiner 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E.K., B.L.v.d.E., A.G., M.H., D.v.B., H.M., M.v.d.B., J.V., R.B.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henri\u00ebt","family":"Meems","sequence":"additional","affiliation":[{"name":"From the Plasma Proteins, Sanquin Research and Landsteiner Laboratory, Academic Medical Center, University of Amsterdam, The Netherlands (M.S., E.K., B.L.v.d.E., A.G., M.H., D.v.B., H.M., M.v.d.B., J.V., R.B.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans","family":"Janssen","sequence":"additional","affiliation":[{"name":"Cell Biology, The Netherlands Cancer Institute, Amsterdam (H.J.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aat A.","family":"Mulder","sequence":"additional","affiliation":[{"name":"Molecular Cell Biology, Section Electron Microscopy, Leiden University Medical Center, The Netherlands (A.A.M., C.R.J., A.J.K.)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolina 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