Therapies in Aicardi-Goutières syndrome.
Clin Exp Immunol
; 175(1): 1-8, 2014 Jan.
Article
en En
| MEDLINE
| ID: mdl-23607857
Aicardi-Goutières syndrome (AGS) is a genetically determined disorder, affecting most particularly the brain and the skin, characterized by the inappropriate induction of a type I interferon-mediated immune response. In most, but not all, cases the condition is severe, with a high associated morbidity and mortality. A number of important recent advances have helped to elucidate the biology of the AGS-related proteins, thus providing considerable insight into disease pathology. In this study, we outline the clinical phenotype of AGS, paying particular attention to factors relevant to therapeutic intervention. We then discuss the pathogenesis of AGS from a molecular and cell biology perspective. Finally, we suggest possible treatment strategies in light of these emerging insights.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Enfermedades Autoinmunes del Sistema Nervioso
/
Malformaciones del Sistema Nervioso
Límite:
Humans
Idioma:
En
Revista:
Clin Exp Immunol
Año:
2014
Tipo del documento:
Article
Pais de publicación:
Reino Unido