Type I interferon–mediated monogenic autoinflammation: The type I interferonopathies, a conceptual overview
نویسندگان
چکیده
منابع مشابه
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...
متن کاملType I interferonopathies in pediatric rheumatology
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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چکیده ندارد.
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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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