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Endoglin prevents v...
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Jin, YiUppsala universitet,Vaskulärbiologi,Karolinska Inst, Dept Med Biochem & Biophys, Scheeles Vag 2, S-17177 Stockholm, Sweden.
(author)
Endoglin prevents vascular malformation by regulating flow-induced cell migration and specification through VEGFR2 signalling
- Article/chapterEnglish2017
Publisher, publication year, extent ...
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2017-05-22
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NATURE PUBLISHING GROUP,2017
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printrdacarrier
Numbers
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LIBRIS-ID:oai:DiVA.org:uu-330738
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https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-330738URI
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https://doi.org/10.1038/ncb3534DOI
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http://kipublications.ki.se/Default.aspx?queryparsed=id:136232708URI
Supplementary language notes
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Language:English
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Summary in:English
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Subject category:ref swepub-contenttype
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Subject category:art swepub-publicationtype
Notes
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Loss-of-function (LOF) mutations in the endothelial cell (EC)-enriched gene endoglin (ENG) cause the human disease hereditary haemorrhagic telangiectasia-1, characterized by vascular malformations promoted by vascular endothelial growth factor A (VEGFA). How ENG deficiency alters EC behaviour to trigger these anomalies is not understood. Mosaic ENG deletion in the postnatal mouse rendered Eng LOF ECs insensitive to flow-mediated venous to arterial migration. Eng LOF ECs retained within arterioles acquired venous characteristics and secondary ENG-independent proliferation resulting in arteriovenous malformation (AVM). Analysis following simultaneous Eng LOF and overexpression (OE) revealed that ENG OE ECs dominate tip-cell positions and home preferentially to arteries. ENG knockdown altered VEGFA-mediated VEGFR2 kinetics and promoted AKT signalling. Blockage of PI(3)K/AKT partly normalized flow-directed migration of ENG LOF ECs in vitro and reduced the severity of AVM in vivo. This demonstrates the requirement of ENG in flow-mediated migration and modulation of VEGFR2 signalling in vascular patterning.
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Muhl, LarsKarolinska Institutet
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Burmakin, MikhailKarolinska Inst, Dept Med Biochem & Biophys, Scheeles Vag 2, S-17177 Stockholm, Sweden.
(author)
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Wang, YixinKarolinska Inst, Dept Med Biochem & Biophys, Scheeles Vag 2, S-17177 Stockholm, Sweden.
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Duchez, Anne-ClaireKarolinska Inst, Dept Med Biochem & Biophys, Scheeles Vag 2, S-17177 Stockholm, Sweden.
(author)
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Betsholtz, ChristerKarolinska Institutet,Uppsala universitet,Vaskulärbiologi,Karolinska Inst, ICMC, Blickagangen 6, SE-14157 Huddinge, Sweden.(Swepub:uu)chbet517
(author)
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Arthur, Helen M.Newcastle Univ, Inst Med Genet, Int Ctr Life, Newcastle Upon Tyne NE1 3BZ, Tyne & Wear, England.
(author)
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Jakobsson, LarsKarolinska Institutet
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Uppsala universitetVaskulärbiologi
(creator_code:org_t)
Related titles
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In:Nature Cell Biology: NATURE PUBLISHING GROUP19:6, s. 639-6521465-73921476-4679
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