Por favor, use este identificador para citar o enlazar a este item:
http://hdl.handle.net/10261/49799
COMPARTIR / EXPORTAR:
SHARE BASE | |
Visualizar otros formatos: MARC | Dublin Core | RDF | ORE | MODS | METS | DIDL | DATACITE | |
Campo DC | Valor | Lengua/Idioma |
---|---|---|
dc.contributor.author | Fernandez-Lopez, Africa | - |
dc.contributor.author | Sanz-Rodríguez, Francisco | - |
dc.contributor.author | Blanco, Francisco J. | - |
dc.contributor.author | Bernabéu, Carmelo | - |
dc.contributor.author | Botella, Luisa María | - |
dc.date.issued | 2006-03-01 | - |
dc.identifier.citation | Clinical Medicine and Research 4 (1) 66-78 (2006) | es_ES |
dc.identifier.issn | 1539-4182 | - |
dc.identifier.uri | http://hdl.handle.net/10261/49799 | - |
dc.description | 13 páginas, 8 figuras, 2 tablas -- PAGS nros. 66-78 | es_ES |
dc.description.abstract | Hereditary hemorrhagic telangiectasia (HHT) is caused by mutations in endoglin (ENG; HHT1) or ACVRL1/ALK1 (HHT2) genes and is an autosomal dominant vascular dysplasia. Clinically, HHT is characterized by epistaxis, telangiectases and arteriovenous malformations in some internal organs such as the lung, brain or liver. Endoglin and ALK1 proteins are specific endothelial receptors of the transforming growth factor (TGF)-β superfamily that are essential for vascular integrity. Genetic studies in mice and humans have revealed the pivotal role of TGF-β signaling during angiogenesis. Through binding to the TGF-β type II receptor, TGF-β can activate two distinct type I receptors (ALK1 and ALK5) in endothelial cells, each one leading to opposite effects on endothelial cell proliferation and migration. The recent isolation and characterization of circulating endothelial cells from HHT patients has revealed a decreased endoglin expression, impaired ALK1- and ALK5-dependent TGF-β signaling, disorganized cytoskeleton and the failure to form cord-like structures which may lead to the fragility of small vessels with bleeding characteristic of HHT vascular dysplasia or to disrupted and abnormal angiogenesis after injuries and may explain the clinical symptoms associated with this disease | es_ES |
dc.description.sponsorship | This work was supported by grants from HHT Foundation International, Ministerio de Educación y Ciencia (SAF2004-01390) and Fondo de Investigación Sanitaria (PI020200) to C.B. A.F-L is a predoctoral fellow of I3P Program from Ministerio de Educación y Ciencia, Spain | es_ES |
dc.language.iso | eng | es_ES |
dc.publisher | Marshfield Clinic | es_ES |
dc.rights | closedAccess | es_ES |
dc.subject | Actin cytoskeleton | es_ES |
dc.subject | ALK1 | es_ES |
dc.subject | Angiogenesis | es_ES |
dc.subject | Endoglin | es_ES |
dc.subject | Endothelial cells | es_ES |
dc.subject | Hereditary hemorrhagic telangiectasia | es_ES |
dc.subject | TGF-β pathway | es_ES |
dc.subject | Vascular dysplasia | es_ES |
dc.title | Hereditary hemorrhagic telangiectasia, a vascular dysplasia affecting the TGF-beta signaling pathway | es_ES |
dc.type | artículo | es_ES |
dc.description.peerreviewed | Peer reviewed | es_ES |
dc.relation.publisherversion | http://doi.org/10.3121/cmr.4.1.66 | es_ES |
dc.identifier.e-issn | 1554-6179 | - |
dc.type.coar | http://purl.org/coar/resource_type/c_6501 | es_ES |
item.openairetype | artículo | - |
item.grantfulltext | none | - |
item.cerifentitytype | Publications | - |
item.openairecristype | http://purl.org/coar/resource_type/c_18cf | - |
item.fulltext | No Fulltext | - |
item.languageiso639-1 | en | - |
Aparece en las colecciones: | (CIB) Artículos |
Ficheros en este ítem:
Fichero | Descripción | Tamaño | Formato | |
---|---|---|---|---|
accesoRestringido.pdf | 15,38 kB | Adobe PDF | Visualizar/Abrir |
CORE Recommender
Page view(s)
300
checked on 22-abr-2024
Download(s)
26
checked on 22-abr-2024
Google ScholarTM
Check
NOTA: Los ítems de Digital.CSIC están protegidos por copyright, con todos los derechos reservados, a menos que se indique lo contrario.