{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T07:37:42Z","timestamp":1791272262633,"version":"4.1.0"},"reference-count":60,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2008,1,31]],"date-time":"2008-01-31T00:00:00Z","timestamp":1201737600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Glia"],"published-print":{"date-parts":[[2008,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Aicardi\u2010Gouti\u00e8res syndrome (AGS) presents as a severe autosomal recessively inherited neurological brain disease. Clinical and neurological manifestations closely resemble those of congenital viral infection and are generally attributed to a perturbation of innate immunity including a long lasting lymphocytosis and production of interferon\u2010alpha (IFN\u03b1) in the central nervous system. To clarify the innate immune response evoked in these diseases, we used a 30\u2010mer multiplexed luminex system to measure multiple cytokines and growth factors in the cerebrospinal fluid and serum of patients with AGS and viral meningitis or encephalitis, and febrile controls in whom infection could not be substantiated. In addition to the previously described IFN\u03b1, both AGS and viral diseases were characterized by expression of CXCL10 and CCL2. In contrast to AGS, viral infection resulted in high levels of IL\u20106 and CXCL8 in the CNS. Postmortem immunohistochemical staining of brain sections showed that in both AGS and viral CNS infection, astrocytes were responsible for the production of cytokines and not the infiltrating leukocytes. In summary, our data indicate that astrocytes are the predominant cell type responsible for the production of IFN\u03b1 and CXCL10 in AGS. Whereas IFN\u03b1 is assumed to be involved in the neurodegeneration, calcifications and seizures in AGS, CXCL10 may act as the chemoattractant responsible for the influx of activated lymphocytes into the brain. The lack of the inflammatory cytokines IL\u20106 and CXCL8 in AGS suggest that the neuroinflammatory reaction in this disease is distinct from viral disease. \u00a9 2008 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/glia.20639","type":"journal-article","created":{"date-parts":[[2008,1,31]],"date-time":"2008-01-31T23:16:18Z","timestamp":1201821378000},"page":"568-578","source":"Crossref","is-referenced-by-count":101,"title":["Astrocytes produce interferon\u2010alpha and CXCL10, but not IL\u20106 or CXCL8, in aicardi\u2010Gouti\u00e8res syndrome"],"prefix":"10.1002","volume":"56","author":[{"given":"Jane T.","family":"Van Heteren","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Flore","family":"Rozenberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleonora","family":"Aronica","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dirk","family":"Troost","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Lebon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taco W.","family":"Kuijpers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2008,1,31]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"crossref","first-page":"5016","DOI":"10.4049\/jimmunol.161.9.5016","article-title":"Transgenic expression of IFN\u2010\u03b1 in the central nervous system of mice protects against lethal neurotropic viral infection but induces inflammation and neurodegeneration","volume":"161","author":"Akwa Y","year":"1998","journal-title":"J Immunol"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.0305-1846.2001.00314.x"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.3109\/13550289909029747"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004010051071"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neulet.2006.11.070"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/glia.10256"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.169.3.1505"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-8993(99)01328-1"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/glia.20117"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/glia.20450"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1080\/13550280500384982"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1080\/13550280152058799"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.176.7.4235"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1067\/mjd.2001.117861"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1845"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1842"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1177\/08830738050200111101"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2005.01.020"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.168.7.3195"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.2003.02202.x"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.3863-05.2006"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2004.09.009"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.166.12.7617"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1189\/jlb.1202598"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.braindev.2003.12.011"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410440608"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M511797200"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.04-3104com"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.175.7.4320"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1080\/13550280600873819"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcb.20052"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-5728(98)00223-9"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00381-006-0112-9"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.21088"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1053\/ejpn.2002.0568"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000159864.05826.08"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1053\/ejpn.2002.0574"},{"key":"e_1_2_7_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(88)90125-6"},{"key":"e_1_2_7_40_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.jid.5700530"},{"key":"e_1_2_7_41_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2990.2000.00243.x"},{"key":"e_1_2_7_42_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.86.16.6348"},{"key":"e_1_2_7_43_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.176.7.4399"},{"key":"e_1_2_7_44_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4615-1325-4_48"},{"key":"e_1_2_7_45_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1521-4141(199811)28:11<3696::AID-IMMU3696>3.0.CO;2-W"},{"key":"e_1_2_7_46_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh655"},{"key":"e_1_2_7_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.siny.2007.01.012"},{"key":"e_1_2_7_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2006.06.018"},{"key":"e_1_2_7_49_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2003.12.025"},{"key":"e_1_2_7_50_1","doi-asserted-by":"publisher","DOI":"10.1096\/fasebj.7.6.8472896"},{"key":"e_1_2_7_51_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2004-830532"},{"key":"e_1_2_7_52_1","doi-asserted-by":"publisher","DOI":"10.1006\/brbi.1995.1034"},{"key":"e_1_2_7_53_1","doi-asserted-by":"publisher","DOI":"10.1086\/513443"},{"key":"e_1_2_7_54_1","doi-asserted-by":"publisher","DOI":"10.1086\/521373"},{"key":"e_1_2_7_55_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2005.01.033"},{"key":"e_1_2_7_56_1","doi-asserted-by":"publisher","DOI":"10.1086\/421244"},{"key":"e_1_2_7_57_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-5728(02)00097-8"},{"key":"e_1_2_7_58_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001756-200110080-00010"},{"key":"e_1_2_7_59_1","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1080\/13550280390201029","article-title":"Adenovirus\u2010mediated expression of CXCL10 in the central nervous system results in T\u2010cell recruitment and limited neuropathology","volume":"9","author":"Trifilo MJ","year":"2003","journal-title":"J Neruovirol"},{"key":"e_1_2_7_60_1","doi-asserted-by":"publisher","DOI":"10.1191\/1352458504ms982oa"},{"key":"e_1_2_7_61_1","doi-asserted-by":"publisher","DOI":"10.1128\/JVI.01592-06"}],"container-title":["Glia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fglia.20639","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/glia.20639","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,14]],"date-time":"2023-11-14T23:12:06Z","timestamp":1700003526000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/glia.20639"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,1,31]]},"references-count":60,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2008,4]]}},"alternative-id":["10.1002\/glia.20639"],"URL":"https:\/\/doi.org\/10.1002\/glia.20639","archive":["Portico"],"relation":{},"ISSN":["0894-1491","1098-1136"],"issn-type":[{"value":"0894-1491","type":"print"},{"value":"1098-1136","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,1,31]]}}}