English   español  
Por favor, use este identificador para citar o enlazar a este item: http://hdl.handle.net/10261/52037
COMPARTIR / IMPACTO:
Estadísticas
logo share SHARE logo core CORE   Add this article to your Mendeley library MendeleyBASE

Visualizar otros formatos: MARC | Dublin Core | RDF | ORE | MODS | METS | DIDL
Exportar a otros formatos:
Título

Blood outgrowth endothelial cells from Hereditary Haemorrhagic Telangiectasia patients reveal abnormalities compatible with vascular lesions

AutorFernandez-Lopez, Africa ; Sanz-Rodriguez, Francisco; Zarrabeitia, Roberto; Pérez-Molino, Alfonso; Hebbeld, Robert P.; Nguyen, Julia; Bernabéu, Carmelo ; Botella, Luisa María
Palabras claveEndothelial receptors
Endothelial funct
Fecha de publicación1-nov-2005
EditorOxford University Press
CitaciónCardiovascular Research 68(2):235-248(2005)
ResumenObjective: Hereditary haemorrhagic telangiectasia (HHT) is originated by mutations in endoglin (HHT1) and ALK1 (HHT2) genes. The purpose of this work was to isolate and characterize circulating endothelial cells from HHT patients. Methods: Pure primary cultures of blood outgrowth endothelial cells (BOECs) were obtained from 50 ml of peripheral blood by selection on collagen plates with endothelial medium. Results: The amount of endoglin in HHT1–BOECs is half the controls, but HHT2–BOECs are also endoglin-deficient. Since the TGF-β/ALK1 pathway activates the endoglin promoter activity, these results suggest the involvement of ALK1 in endoglin gene expression. Endothelial TGF-β pathways, mediated by ALK1 and ALK5, are impaired in HHT cells. HHT–BOECs show disorganized and depolymerized actin fibers, as compared to the organized stress fibers of healthy–BOECs. Functionally, HHT–BOECs have impaired tube formation, in contrast with the cord-like structures derived from normal donors. Conclusions: Decreased endoglin expression, impaired TGF-β signalling, disorganized cytoskeleton, and failure to form cord-like structures are common characteristics of endothelial cells from HHT patients. These features may lead to fragility of small vessels and bleeding characteristic of the HHT vascular dysplasia and to a disrupted and abnormal angiogenesis, which may explain the clinical symptoms associated with this disease
Descripción14 páginas, 9 figuras -- PAGS nros. 235-248
Versión del editorhttp:dx.doi.org/10.1016/j.cardiores.2005.06
URIhttp://hdl.handle.net/10261/52037
DOI10.1016/j.cardiores.2005.06.009
ISSN0008-6363
E-ISSN1755-3245
Aparece en las colecciones: (CIB) Artículos
Ficheros en este ítem:
No hay ficheros asociados a este ítem.
Mostrar el registro completo
 

Artículos relacionados:


NOTA: Los ítems de Digital.CSIC están protegidos por copyright, con todos los derechos reservados, a menos que se indique lo contrario.