Type I interferon-mediated monogenic autoinflammation: The type I interferonopathies, a conceptual overview.

ABSTRACT: Type I interferon is a potent substance. As such, the induction, transmission, and resolution of the type I interferon-mediated immune response are tightly regulated. As defined, the type I interferonopathies represent discrete examples of a disturbance of the homeostatic control of this system caused by Mendelian mutations. Considering the complexity of the interferon response, the identification of further monogenic diseases belonging to this disease grouping seems likely, with the recognition of type I interferonopathies becoming of increasing clinical importance as treatment options are developed based on an understanding of disease pathology and innate immune signaling. Definition of the type I interferonopathies indicates that autoinflammation can be both interferon and noninterferon

SUBMITTER: Rodero MP 

PROVIDER: S-EPMC5110029 | biostudies-literature | 2016 Nov

REPOSITORIES: biostudies-literature