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Type I interferon-mediated monogenic autoinflammation: The type I
interferonopathies, a conceptual overview
#MMPMID27821552
Rodero MP
; Crow YJ
J Exp Med
2016[Nov]; 213
(12
): 2527-2538
PMID27821552
show ga
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 related, and that a primary disturbance of the innate immune
system can "spill over" into autoimmunity in some cases. Indeed, that several
non-Mendelian disorders, most particularly systemic lupus erythematosus and
dermatomyositis, are also characterized by an up-regulation of type I interferon
signaling suggests the possibility that insights derived from this work will have
relevance to a broader field of clinical medicine.
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