Journal
SEMINARS IN IMMUNOPATHOLOGY
Volume 37, Issue 4, Pages 349-357Publisher
SPRINGER HEIDELBERG
DOI: 10.1007/s00281-015-0500-x
Keywords
Type I interferonopathies; Type I interferon; Autoinflammation; Autoimmunity; Aicardi-Goutieres syndrome; Familial chilblain lupus; Systemic lupus erythematosus
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Funding
- Deutsche Forschungsgemeinsschaft (Clinical Research Group) [249]
- Friede Springer Stiftung
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Type I interferons (IFNs) play a central role in the immune defense against viral infections. Type I IFN signaling is activated by pattern recognition receptors upon sensing of viral nucleic acids and induces antiviral programs through modulation of innate and adaptive immune responses. Type I interferonopathies comprise a heterogenous group of genetically determined diseases that are characterized by inappropriate activation of type I IFN. While their phenotypic spectrum is broad, ranging from severe neurological impairment to mild cutaneous disease, systemic autoinflammation, and autoimmunity are commonly shared signs of type I interferonopathies. Although the mechanisms underlying various disease phenotypes associated with inappropriate type I IFN activation have yet to be fully elucidated, our current understanding of the molecular pathogenesis of type I interferonopathies has provided a set of candidate molecules that can be interrogated in search of targeted therapies.
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