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Seminars in Immunopathology
Published in: Seminars in Immunopathology 4/2015

01-07-2015 | Review

Type I interferonopathies—an expanding disease spectrum of immunodysregulation

Authors: Min Ae Lee-Kirsch, Christine Wolf, Stefanie Kretschmer, Axel Roers

Published in: Seminars in Immunopathology | Issue 4/2015

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Abstract

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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Metadata
Title
Type I interferonopathies—an expanding disease spectrum of immunodysregulation
Authors
Min Ae Lee-Kirsch
Christine Wolf
Stefanie Kretschmer
Axel Roers
Publication date
01-07-2015
Publisher
Springer Berlin Heidelberg
Published in
Seminars in Immunopathology / Issue 4/2015
Print ISSN: 1863-2297
Electronic ISSN: 1863-2300
DOI
https://doi.org/10.1007/s00281-015-0500-x

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