Journal article

An Update on Autoinflammatory Diseases: Interferonopathies

Sophia Davidson, Annemarie Steiner, Cassandra R Harapas, Seth L Masters

CURRENT RHEUMATOLOGY REPORTS | SPRINGER | Published : 2018

Abstract

PURPOSE OF REVIEW: Type I interferons (IFNαβ) induce the expression of hundreds of genes; thus, it is unsurprising that the initiation, transmission, and resolution of the IFNαβ-mediated immune response is tightly controlled. Mutations that alter nucleic acid processing and recognition, ablate IFNαβ-specific negative feedback mechanisms, or result in dysfunction of the proteasome system can all induce pathogenic IFNαβ signalling and are the focus of this review. RECENT FINDINGS: Recent advances have delineated the precise cytoplasmic mechanisms that facilitate self-DNA to be recognised by cGAS and self-RNA to be recognised by RIG-I or MDA-5. This helps clarify interferonopathies associated w..

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