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

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

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 identificat...

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Defective regulation of type I interferon response is associated with severe inflammatory phenotypes and autoimmunity. Type I interferonopathies are a clinically heterogenic group of Mendelian diseases with a constitutive activation of this pathway that might present as atypical, severe, early onset rheumatic diseases. Skin vasculopathy with chilblains and livedo reticularis, interstitial lung ...

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The Type I Generalized Half Logistic Distribution

In this paper, we considered the half logistic model and derived a probability density function that generalized it. The cumulative distribution function, the $n^{th}$ moment, the median, the mode and the 100$k$-percentage points of the generalized distribution were established. Estimation of the parameters of the distribution through maximum likelihood method was accomplished with the aid of c...

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Identification of type I interferonopathies using blood interferon signature: the experience of a pediatric rheumatology center

Methods We collected blood samples from a cohort of pediatric rheumatologic patients and scored them according to a qPCR based IFN gene signature assay. Expression of 6 type I IFN-related genes (IFI27, IFI44L, IFIT1, ISG15, RSAD2, SIGLEC1) was quantified by standard RTPCR techniques. An IFN score was calculated for each patient using the median fold change of gene expression related to a health...

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ژورنال

عنوان ژورنال: Journal of Experimental Medicine

سال: 2016

ISSN: 0022-1007,1540-9538

DOI: 10.1084/jem.20161596