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Therapies in Aicardi-Goutières syndrome.
- Source :
-
Clinical and experimental immunology [Clin Exp Immunol] 2014 Jan; Vol. 175 (1), pp. 1-8. - Publication Year :
- 2014
-
Abstract
- 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.<br /> (© 2013 British Society for Immunology.)
- Subjects :
- Humans
Interferon Type I genetics
Interferon Type I immunology
Autoimmune Diseases of the Nervous System genetics
Autoimmune Diseases of the Nervous System immunology
Autoimmune Diseases of the Nervous System pathology
Autoimmune Diseases of the Nervous System therapy
Nervous System Malformations genetics
Nervous System Malformations immunology
Nervous System Malformations pathology
Nervous System Malformations therapy
Subjects
Details
- Language :
- English
- ISSN :
- 1365-2249
- Volume :
- 175
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Clinical and experimental immunology
- Publication Type :
- Academic Journal
- Accession number :
- 23607857
- Full Text :
- https://doi.org/10.1111/cei.12115