Aicardi–Goutières syndrome and the type I interferonopathies

@article{Crow2015AicardiGoutiresSA,
  title={Aicardi–Gouti{\`e}res syndrome and the type I interferonopathies},
  author={Yanick J. Crow and Nicolas Manel},
  journal={Nature Reviews Immunology},
  year={2015},
  volume={15},
  pages={429-440}
}
Dissection of the genetic basis of Aicardi–Goutières syndrome has highlighted a fundamental link between nucleic acid metabolism, innate immune sensors and type I interferon induction. This had led to the concept of the human interferonopathies as a broader set of Mendelian disorders in which a constitutive upregulation of type I interferon activity directly relates to disease pathology. Here, we discuss the molecular and cellular basis of the interferonopathies, their categorization, future… 
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