Sökning: WFRF:(Link K.) > (2005-2009) > Interferon autoanti...
Fältnamn | Indikatorer | Metadata |
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000 | 03474naa a2200541 4500 | |
001 | oai:DiVA.org:uu-17670 | |
003 | SwePub | |
008 | 080812s2008 | |||||||||||000 ||eng| | |
024 | 7 | a https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-176702 URI |
024 | 7 | a https://doi.org/10.1182/blood-2008-03-1446342 DOI |
040 | a (SwePub)uu | |
041 | a engb eng | |
042 | 9 SwePub | |
072 | 7 | a ref2 swepub-contenttype |
072 | 7 | a art2 swepub-publicationtype |
100 | 1 | a Kisand, Kaiu Uppsala universitet,Institutionen för medicinska vetenskaper,Autoimmuna sjukdomar (Kämpe)4 aut |
245 | 1 0 | a Interferon autoantibodies associated with AIRE-deficiency decrease the expression of IFN-stimulated genes |
264 | 1 | b American Society of Hematology,c 2008 |
338 | a print2 rdacarrier | |
520 | a Neutralizing autoantibodies to type I, but not type II, interferons (IFNs) are found at high titers in almost every patient with autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED), a disease caused by AIRE gene mutations that lead to defects in thymic T-cell selection. Combining genome-wide expression array with real time RT-PCR assays, we here demonstrate that antibodies against IFN-alpha cause highly significant down-regulation of interferon-stimulated gene expression in cells from APECED patients' blood by blocking their highly dilute endogenous IFNs. This down-regulation was lost progressively as these APECED cells matured in cultures without neutralizing autoantibodies. Most interestingly, a rare APECED patient with autoantibodies to IFN-omega but not IFN-alpha showed a marked increase in expression of the same interferon-stimulated genes. We also report unexpected increases in serum CXCL10 levels in APECED. Our results argue that the breakdown of tolerance to IFNs in AIRE deficiency is associated with impaired responses to them in thymus, and highlight APECED as another autoimmune disease with associated dysregulation of IFN activity. | |
653 | a MEDICINE | |
653 | a MEDICIN | |
700 | 1 | a Link, Maire4 aut |
700 | 1 | a Wolff, Anette S Bu Uppsala universitet,Medicin,Autoimmuna sjukdomar (Kämpe)4 aut |
700 | 1 | a Meager, Anthony4 aut |
700 | 1 | a Tserel, Liina4 aut |
700 | 1 | a Org, Tonis4 aut |
700 | 1 | a Murumagi, Astrid4 aut |
700 | 1 | a Uibo, Raivo4 aut |
700 | 1 | a Willcox, Nick4 aut |
700 | 1 | a Trebusak Podkrajsek, Katarina4 aut |
700 | 1 | a Battelino, Tadej4 aut |
700 | 1 | a Lobell, Annau Uppsala universitet,Institutionen för medicinska vetenskaper,Autoimmuna sjukdomar (Kämpe)4 aut0 (Swepub:uu)anlob030 |
700 | 1 | a Kämpe, Olleu Uppsala universitet,Institutionen för medicinska vetenskaper,Autoimmuna sjukdomar (Kämpe)4 aut0 (Swepub:uu)ollekamp |
700 | 1 | a Lima, Kari4 aut |
700 | 1 | a Meloni, Antonella4 aut |
700 | 1 | a Ergun-Longmire, Berrin4 aut |
700 | 1 | a Maclaren, Noel K4 aut |
700 | 1 | a Perheentupa, Jaakko4 aut |
700 | 1 | a Krohn, Kai J E4 aut |
700 | 1 | a Scott, Hamish S4 aut |
700 | 1 | a Husebye, Eystein S4 aut |
700 | 1 | a Peterson, Part4 aut |
710 | 2 | a Uppsala universitetb Institutionen för medicinska vetenskaper4 org |
773 | 0 | t Bloodd : American Society of Hematologyg 112:7, s. 2657-2666q 112:7<2657-2666x 0006-4971x 1528-0020 |
856 | 4 | u https://europepmc.org/articles/pmc2577576 |
856 | 4 8 | u https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-17670 |
856 | 4 8 | u https://doi.org/10.1182/blood-2008-03-144634 |
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