Reverse-Transcriptase Inhibitors in the Aicardi–Goutières Syndrome - Institut Pasteur Access content directly
Journal Articles New England Journal of Medicine Year : 2018

Reverse-Transcriptase Inhibitors in the Aicardi–Goutières Syndrome

Gillian Rice
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Nathalie Boddaert
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Flore Rozenberg
Vincent Bondet
Darragh Duffy
Alexandre Belot
Diane Doummar
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Florence Renaldo
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Diana Rodriguez
Luis Seabra
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Stéphane Blanche
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Yanick Crow
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To the Editor: The Aicardi–Goutières syndrome is a genetic encephalopathy that is associated with childhood illness and death. The syndrome is hypothesized to be due to misidentification of self-derived nucleic acids as nonself and the subsequent induction of a type I interferon–mediated response that simulates an antiviral reaction.1 Endogenous retroelements, mobile genetic elements that can be transcribed to RNA and then to DNA by reverse transcription, constitute 40% of the human genome and represent a potential source of immunostimulatory nucleic acid in patients with this syndrome.2


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pasteur-01974160 , version 1 (08-01-2019)



Gillian Rice, Candice Meyzer, Naïm Bouazza, Marie Hully, Nathalie Boddaert, et al.. Reverse-Transcriptase Inhibitors in the Aicardi–Goutières Syndrome. New England Journal of Medicine, 2018, 379 (23), pp.2275-2277. ⟨10.1056/NEJMc1810983⟩. ⟨pasteur-01974160⟩
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