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Blood, 1 March 2008, Vol. 111, No. 5, pp. 2657-2666. Prepublished online as a Blood First Edition Paper on December 19, 2007; DOI 10.1182/blood-2007-07-100412. This Article Full Text Full Text (PDF) Supplemental Tables, Figures, and Videos All Versions of this Article: blood-2007-07-100412v1 111/5/2657    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Cermenati, S. Articles by Beltrame, M. Search for Related Content PubMed PubMed Citation Articles by Cermenati, S. Articles by Beltrame, M. Related Collections Hemostasis, Thrombosis, and Vascular Biology Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Sox18 and Sox7 play redundant roles in vascular development Solei Cermenati1, Silvia Moleri1, Simona Cimbro2, Paola Corti1, Luca Del Giacco2, Roberta Amodeo1, Elisabetta Dejana1,3, Peter Koopman4, Franco Cotelli2, and Monica Beltrame1 1 Dipartimento di Scienze Biomolecolari e Biotecnologie, Università degli Studi di Milano, Milan, Italy; 2 Dipartimento di Biologia, Università degli Studi di Milano, Milan, Italy; 3 FIRC Institute of Molecular Oncology (IFOM), Milan, Italy; and 4 Institute for Molecular Bioscience, The University of Queensland, Brisbane, Australia Mutations in SOX18 cause the human hypotrichosis-lymphedema-telangiectasia (HLT) syndrome. Their murine counterparts are the spontaneous ragged mutants, showing combined defects in hair follicle, blood vessel, and lymphatic vessel development. Mice null for Sox18 display only mild coat defects, suggesting a dominant-negative effect of Sox18/ragged mutations and functional redundancy between Sox18 and other Sox-F proteins. We addressed this point in zebrafish. The zebrafish homologs of Sox18 and of Sox7 are expressed in angioblasts and in the endothelial component of nascent blood vessels in embryos. Knockdown of either gene, using moderate doses of specific morpholinos, had minimal effects on vessels. In contrast, simultaneous knockdown of both genes resulted in multiple fusions between the major axial vessels. With combined use of transgenic lines and molecular markers, we could show that endothelial cells are specified, but fail to acquire a correct arteriovenous identity. Venous endothelial cell differentiation was more severely affected than arterial. Thus, sox7 and sox18 play redundant but collectively essential roles in the establishment of proper arteriovenous identity in zebrafish. Our data suggest that a defect in arteriovenous identity could be responsible for the formation of telangiectases in patients with HLT. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: S. P. Herbert, J. Huisken, T. N. Kim, M. E. Feldman, B. T. Houseman, R. A. Wang, K. M. Shokat, and D. Y. R. Stainier Arterial-Venous Segregation by Selective Cell Sprouting: An Alternative Mode of Blood Vessel Formation Science, October 9, 2009; 326(5950): 294 - 298. [Abstract] [Full Text] [PDF] M. Downes, M. Francois, C. Ferguson, R. G. Parton, and P. Koopman Vascular defects in a mouse model of hypotrichosis-lymphedema-telangiectasia syndrome indicate a role for SOX18 in blood vessel maturation Hum. Mol. Genet., August 1, 2009; 18(15): 2839 - 2850. [Abstract] [Full Text] [PDF] B. Hosking, M. Francois, D. Wilhelm, F. Orsenigo, A. Caprini, T. Svingen, D. Tutt, T. Davidson, C. Browne, E. Dejana, et al. Sox7 and Sox17 are strain-specific modifiers of the lymphangiogenic defects caused by Sox18 dysfunction in mice Development, July 15, 2009; 136(14): 2385 - 2391. [Abstract] [Full Text] [PDF] N. Limaye, L. M. Boon, and M. Vikkula From germline towards somatic mutations in the pathophysiology of vascular anomalies Hum. Mol. Genet., April 15, 2009; 18(R1): R65 - R74. [Abstract] [Full Text] [PDF] M. R. Swift and B. M. Weinstein Arterial-Venous Specification During Development Circ. Res., March 13, 2009; 104(5): 576 - 588. [Abstract] [Full Text] [PDF]

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