Type I interferons (IFNs) as part of the innate immune system have an outstanding
importance as antiviral defense cytokines that stimulate innate and adaptive immune
responses. Upon sensing of pattern recognition particles (PRPs) such as nucleic acids,
IFN secretion is activated and induces the expression of interferon stimulated genes
(ISGs). Uncontrolled constitutive activation of the type I IFN system can lead to
autoinflammation and autoimmunity, which is observed in autoimmune disorders such
as systemic lupus erythematodes and in monogenic interferonopathies. They are caused
by mutations in genes which are involved in sensing or metabolism of intracellular nucleic
acids and DNA repair. Many authors described mechanisms of type I IFN secretion upon
increased DNA damage, including the formation of micronuclei, cytosolic chromatin
fragments and destabilization of DNA binding proteins. Hereditary cutaneous DNA
damage syndromes, which are caused by mutations in proteins of the DNA repair,
share laboratory and clinical features also seen in autoimmune disorders and
interferonopathies; hence a potential role of DNA-damage-induced type I IFN secretion
seems likely. Here, we aim to summarize possible mechanisms of IFN induction in
cutaneous DNA damage syndromes with defects in the DNA double-strand repair and
nucleotide excision repair. We review recent publications referring to Ataxia
teleangiectasia, Bloom syndrome, Rothmund–Thomson syndrome, Werner syndrome,
Huriez syndrome, and Xeroderma pigmentosum. Furthermore, we aim to discuss the role
of type I IFN in cancer and these syndromes.
| Identifer | oai:union.ndltd.org:DRESDEN/oai:qucosa:de:qucosa:84306 |
| Date | 24 March 2023 |
| Creators | Klein, Benjamin, Günther, Claudia |
| Publisher | Frontiers Research Foundation |
| Source Sets | Hochschulschriftenserver (HSSS) der SLUB Dresden |
| Language | English |
| Detected Language | English |
| Type | info:eu-repo/semantics/publishedVersion, doc-type:article, info:eu-repo/semantics/article, doc-type:Text |
| Rights | info:eu-repo/semantics/openAccess |
| Relation | 1664-3224, 715723 |
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