Your browser doesn't support javascript.

Portal de Búsqueda de la BVS

Información y Conocimiento para la Salud

Home > Búsqueda > ()
Imprimir Exportar

Formato de exportación:


Adicionar mas contactos
| |

Sindrome linfoproliferativo ligado al cromosoma X, infeccion por el virus EBV y defectos en la regulacion de la citotoxicidad linfocitaria / X-linked lymphoproliferative syndrome, EBV virus infection and defects in cytotoxicity lymphocyte regulation

Malbran, Alejandro; Belmonte, Liliana; Ruibal-Ares, Beatriz; Bare, Patricia; Bracco, Maria Marta E.
Medicina (B.Aires) ; 63(1): 70-76, 2003. ilus
Artículo en Español | LILACS | ID: lil-334553
Mutations in SH2D1A, a gene that codifies for the regulatory protein SAP, result in uncontrolled activation of the SLAM (signaling lymphocyte-activation molecule) pathway. This X-linked immunodeficiency becomes evident when the patients are infected with Epstein Barr virus (EBV) and develop a fulminant form of infectious mononucleosis leading to a lymphoproliferative syndrome that is often fatal (X-linked lymphoproliferative syndrome, XLP). In those who survive, hypogammaglobulinemia and oncohematologic diseases are frequently observed. In this revision, the immuno-regulatory mechanisms involved in XLP immunopathology and the role of different effector cells (CD8 T lymphocytes, NK cells) are discussed
Biblioteca responsable: BR1.1