Approaching the Asymptote? Evolution and Revolution in Immunology, Cold Spring Harbor Symposia on Quantitative Biology, vol.54, issue.0, pp.1-13, 1989. ,
DOI : 10.1101/SQB.1989.054.01.003
Nucleic Acid Immunity, Adv. Immunol, vol.133, pp.121-169, 2017. ,
DOI : 10.1016/bs.ai.2016.11.001
Intracellular Nucleic Acid Detection in Autoimmunity, Annual Review of Immunology, vol.35, issue.1, pp.313-336, 2017. ,
DOI : 10.1146/annurev-immunol-051116-052331
Discriminating self from non-self in nucleic acid sensing, Nature Reviews Immunology, vol.516, issue.9, pp.566-580, 2016. ,
DOI : 10.1038/cr.2007.81
Recognition of Endogenous Nucleic Acids by the Innate Immune System, Immunity, vol.44, issue.4, pp.739-754, 2016. ,
DOI : 10.1016/j.immuni.2016.04.002
Type I interferonopathies: a novel set of inborn errors of immunity, Annals of the New York Academy of Sciences, vol.135, issue.1 ,
DOI : 10.1309/AJCP66QIMFARLZKI
Type I interferon???mediated monogenic autoinflammation: The type I interferonopathies, a conceptual overview, The Journal of Experimental Medicine, vol.12, issue.12 ,
DOI : 10.1038/nature13801
Mutations in the gene encoding the 3???-5??? DNA exonuclease TREX1 cause Aicardi-Gouti??res syndrome at the AGS1 locus, Nature Genetics, vol.117, issue.Suppl., pp.917-920, 2006. ,
DOI : 10.1002/ijc.21402
Trex1 Prevents Cell-Intrinsic Initiation of Autoimmunity, Cell, vol.134, issue.4, pp.587-598, 2008. ,
DOI : 10.1016/j.cell.2008.06.032
Intrinsic Self-DNA Triggers Inflammatory Disease Dependent on STING, The Journal of Immunology, vol.193, issue.9, pp.4634-4642, 2014. ,
DOI : 10.4049/jimmunol.1401337
Cutting Edge: cGAS Is Required for Lethal Autoimmune Disease in the Trex1-Deficient Mouse Model of Aicardi???Gouti??res Syndrome, The Journal of Immunology, vol.195, issue.5, pp.1939-1943, 2015. ,
DOI : 10.4049/jimmunol.1500969
Activation of cyclic GMP-AMP synthase by self-DNA causes autoimmune diseases, Proc. Natl Acad. Sci. USA, pp.5699-5705, 2015. ,
DOI : 10.1172/JCI23587
Assessment of interferon-related biomarkers in Aicardi-Gouti??res syndrome associated with mutations in TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, and ADAR: a case-control study, The Lancet Neurology, vol.12, issue.12, pp.1159-1169, 2013. ,
DOI : 10.1016/S1474-4422(13)70258-8
Assessment of Type I Interferon Signaling in Pediatric Inflammatory Disease, Journal of Clinical Immunology, vol.5, issue.5, pp.123-132, 2017. ,
DOI : 10.1016/j.cyto.2015.08.045
DNA Degradation and Its Defects, Cold Spring Harbor Perspectives in Biology, vol.6, issue.6, p.16394, 2014. ,
DOI : 10.1101/cshperspect.a016394
URL : http://cshperspectives.cshlp.org/content/6/6/a016394.full.pdf
Listening to silence and understanding nonsense: exonic mutations that affect splicing, Nature Reviews Genetics, vol.63, issue.Suppl. 1, pp.285-298, 2002. ,
DOI : 10.1086/302119
Human lysosomal DNase II?? contains two requisite PLD-signature (HxK) motifs: Evidence for a pseudodimeric structure of the active enzyme species, Protein Science, vol.16, issue.1, pp.82-91, 2007. ,
DOI : 10.1110/ps.062535307
Requirement of DNase II for Definitive Erythropoiesis in the Mouse Fetal Liver, Science, vol.292, issue.5521, pp.1546-1549, 2001. ,
DOI : 10.1126/science.292.5521.1546
Deoxyribonuclease II?? is required during the phagocytic phase of apoptosis and its loss causes perinatal lethality, Cell Death & Differentiation, vol.255, issue.9, pp.956-962, 2002. ,
DOI : 10.1006/abio.1997.2496
Lethal anemia caused by interferon-?? produced in mouse embryos carrying undigested DNA, Nature Immunology, vol.271, issue.1, pp.49-56, 2005. ,
DOI : 10.1074/jbc.271.1.486
Chronic polyarthritis caused by mammalian DNA that escapes from degradation in macrophages, Nature, vol.8, issue.7114, pp.998-1002, 2006. ,
DOI : 10.1023/A:1008994831937
Cytokinedependent but acquired immunity-independent arthritis caused by DNA escaped from degradation, Proc. Natl Acad. Sci. USA, pp.19432-19437, 2010. ,
STING manifests self DNAdependent inflammatory disease, Proc. Natl Acad. Sci. USA, pp.19386-19391, 2012. ,
DOI : 10.1073/pnas.1215006109
URL : http://www.pnas.org/content/109/47/19386.full.pdf
AIRE-Deficient Patients Harbor Unique High-Affinity Disease-Ameliorating Autoantibodies, Cell, vol.166, issue.3, pp.582-595, 2016. ,
DOI : 10.1016/j.cell.2016.06.024
URL : https://doi.org/10.1016/j.cell.2016.06.024
Single-molecule enzyme-linked immunosorbent assay detects serum proteins at subfemtomolar concentrations, Nature Biotechnology, vol.8, issue.6, pp.595-599, 2010. ,
DOI : 10.1038/nbt.1641
URL : http://europepmc.org/articles/pmc2919230?pdf=render
Detection of interferon alpha protein reveals differential levels and cellular sources in disease, The Journal of Experimental Medicine, vol.31, issue.5, pp.1547-1555, 2017. ,
DOI : 10.1161/STROKEAHA.116.014330
URL : https://hal.archives-ouvertes.fr/pasteur-01534181
Phosphatidylserine-dependent engulfment by macrophages of nuclei from erythroid precursor cells, Nature, vol.15, issue.7059, pp.754-758, 2005. ,
DOI : 10.1359/jbmr.2000.15.8.1477
Erythroblastic islands: niches for erythropoiesis, Blood, vol.112, issue.3, pp.470-478, 2008. ,
DOI : 10.1182/blood-2008-03-077883
DNase II: genes, enzymes and function, Gene, vol.322, pp.1-15, 2003. ,
DOI : 10.1016/j.gene.2003.08.022
Autoinflammation by Endogenous DNA, Adv. Immunol, vol.110, pp.139-161, 2011. ,
DOI : 10.1016/B978-0-12-387663-8.00004-1
IFN regulatory factor (IRF) 3/7-dependent and -independent gene induction by mammalian DNA that escapes degradation, European Journal of Immunology, vol.15, issue.11, pp.3150-3158, 2008. ,
DOI : 10.1038/sj.cdd.4401056
Clinical and Molecular Phenotype of Aicardi-Gouti??res Syndrome, The American Journal of Human Genetics, vol.81, issue.4 ,
DOI : 10.1086/521373
Human USP18 deficiency underlies type 1 interferonopathy leading to severe pseudo-TORCH syndrome, The Journal of Experimental Medicine, vol.213, issue.7, pp.1163-1174, 2016. ,
DOI : 10.1038/nature13801
Virus-induced Transient Bone Marrow Aplasia: Major Role of Interferon-??/?? during Acute Infection with the Noncytopathic Lymphocytic Choriomeningitis Virus, The Journal of Experimental Medicine, vol.48, issue.3 ,
DOI : 10.4269/ajtmh.1993.48.222
Musculoskeletal Disease in MDA5-Related Type I Interferonopathy: A Mendelian Mimic of Jaccoud's Arthropathy, Arthritis & Rheumatology, vol.138, issue.10, pp.2081-2091, 2017. ,
DOI : 10.1016/j.jaci.2016.07.015
Immune Interferon in the Circulation of Patients with Autoimmune Disease, New England Journal of Medicine, vol.301, issue.1, pp.5-8, 1979. ,
DOI : 10.1056/NEJM197907053010102
Features of systemic lupus erythematosus in Dnase1-deficient mice, Nat. Genet, vol.25, pp.177-181, 2000. ,
Mutations in the gene encoding the 3???-5??? DNA exonuclease TREX1 are associated with systemic lupus erythematosus, Nature Genetics, vol.39, issue.9 ,
DOI : 10.1038/nature05245
Loss-of-function variant in DNASE1L3 causes a familial form of systemic lupus erythematosus, Nature Genetics, vol.43, issue.12, pp.1186-1188, 2011. ,
DOI : 10.1038/gene.2009.1
Digestion of Chromatin in Apoptotic Cell Microparticles Prevents Autoimmunity, Cell, vol.166, issue.1, pp.88-101, 2016. ,
DOI : 10.1016/j.cell.2016.05.034
FOREIGN NUCLEIC ACIDS AS THE STIMULUS TO MAKE INTERFERON, The Lancet, vol.282, issue.7299, pp.113-116, 1963. ,
DOI : 10.1016/S0140-6736(63)92585-6
Efficacy of the Janus kinase 1/2 inhibitor ruxolitinib in the treatment of vasculopathy associated with TMEM173 -activating mutations in 3 children, Journal of Allergy and Clinical Immunology, vol.138, issue.6, pp.1752-1755, 2016. ,
DOI : 10.1016/j.jaci.2016.07.015
Brief Report: Blockade of TANK-Binding Kinase 1/IKK?? Inhibits Mutant Stimulator of Interferon Genes (STING)-Mediated Inflammatory Responses in Human Peripheral Blood Mononuclear Cells, Arthritis & Rheumatology, vol.195, issue.7, pp.1495-1501, 2017. ,
DOI : 10.4049/jimmunol.1500162
Translating nucleic acid-sensing pathways into therapies, Nature Reviews Immunology, vol.9, issue.9, pp.529-544, 2015. ,
DOI : 10.1016/j.celrep.2014.10.041
Enzymatic assembly of DNA molecules up to several hundred kilobases, Nature Methods, vol.102, issue.5, pp.343-345, 2009. ,
DOI : 10.1038/nmeth.1318
Production, concentration and titration of pseudotyped HIV-1-based lentiviral vectors, Nature Protocols, vol.70, issue.4, pp.495-505, 2009. ,
DOI : 10.1038/sj.gt.3302189
Restriction by SAMHD1 limits cGAS/STING-dependent innate and adaptive immune responses to HIV-1. Cell Rep, pp.1492-1501, 2016. ,
Deoxyribonuclease II is a lysosomal barrier to transfection, Molecular Therapy, vol.8, issue.6, pp.957-963, 2003. ,
DOI : 10.1016/j.ymthe.2003.09.011
CRYSTALLINE DESOXYRIBONUCLEASE: I. ISOLATION AND GENERAL PROPERTIES SPECTROPHOTOMETRIC METHOD FOR THE MEASUREMENT OF DESOXYRIBONUCLEASE ACTIVITY, The Journal of General Physiology, vol.33, issue.4, pp.349-362, 1950. ,
DOI : 10.1085/jgp.33.4.349
Structure-Function Analysis of STING Activation by c[G(2???,5???)pA(3???,5???)p] and Targeting by Antiviral DMXAA, Cell, vol.154, issue.4, pp.748-762, 2013. ,
DOI : 10.1016/j.cell.2013.07.023
Recognition of 5??? Triphosphate by RIG-I Helicase Requires Short Blunt Double-Stranded RNA as Contained in Panhandle of Negative-Strand Virus, Immunity, vol.31, issue.1, pp.25-34, 2009. ,
DOI : 10.1016/j.immuni.2009.05.008
HSP70 sequestration by free ??-globin promotes ineffective erythropoiesis in ??-thalassaemia, Nature, vol.240, issue.7521, pp.242-246, 2014. ,
DOI : 10.1085/jgp.13.4.469
Structural requirements of human DNase IIalpha for formation of the active enzyme: the role of the signal peptide, N-glycosylation, and disulphide bridging, Biochemical Journal, vol.371, issue.3, pp.867-876, 2003. ,
DOI : 10.1042/bj20021875
Naoki Kitabayashi 1 , Fabrice Porcheray 5 , Frederic Rieux-Laucat 12,13 , Luis Seabra 1 , Carolina Uggenti 1, Nathalie Boddaert Sophie Candon Bénédicte Neven Patrick Nitschke, vol.171720221320323334171318133637412, issue.13, p.3131313138 ,
Laboratory of Cellular and Molecular Mechanisms of Hematological Disorders and Therapeutical Implications, Imagine Institute, Universite?ParisUniversite?Paris Descartes, Sorbonne Paris-Citeét Assistance publique-Ho? pitaux de Paris, Ho? pital Necker Laboratory of Excellence GR-ex, Paris 75015, France. 11 Unita' Operativa Pediatria 2, Istituto Giannina Gaslini, Genova 16147, Italy. 12 Paris Descartes University, Sorbonne-Paris-Cité, Institut Imagine, Paris 75015, France. 13 Laboratory of Immunogenetics of Pediatric Autoimmunity, INSERM UMR 1163 16 Service d'endocrinologie, Gynécologie et Diabétologie Pédiatriques, 18 Institute for Maternal and Child Health-IRCCS " Burlo Garofolo 25 Department of Advanced Diagnostic and Clinical Trials, Institute for Maternal and Child Health-IRCCS " Burlo Garofolo Pediatric Immunology-Hematology ARTICLE NATURE COMMUNICATIONS | DOI, pp.28-29, 1038. ,
37 Service d'he?matologiehe?matologie, Faculte?deFaculte?de Me?decineMe?decine Paris Descartes, Sorbonne Paris-Citeét Assistance Publique- Ho? pitaux de Paris Ho? pital Necker, Paris 75015, France. 38 Department of Genetics, Hôpital Necker-Enfants Malades Alessandra Tesser and Eva Bartok contributed equally to this work. Brigitte Bader-Meunier and Alberto Tommasini jointly supervised this work, NATURE COMMUNICATIONS ,