{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,24]],"date-time":"2026-04-24T03:13:07Z","timestamp":1777000387343,"version":"3.51.4"},"reference-count":55,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2015,9,30]],"date-time":"2015-09-30T00:00:00Z","timestamp":1443571200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"association des malades du syndrome n\u00e9phrotique","award":["nephrochain"],"award-info":[{"award-number":["nephrochain"]}]},{"DOI":"10.13039\/501100003386","name":"soci\u00e9t\u00e9 de n\u00e9phrologie","doi-asserted-by":"crossref","award":["nephroIg"],"award-info":[{"award-number":["nephroIg"]}],"id":[{"id":"10.13039\/501100003386","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2015,11,2]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:p>The efficacy of steroids and immunosuppressive treatments in idiopathic nephrotic syndrome (INS) hints at the implication of immune cells in the pathophysiology of the disease. Toll-like receptor (TLR) dysfunctions are involved in many kidney diseases of immune origin, but remain little described in INS. We investigated the expression and function of TLRs in peripheral blood mononuclear cells (PBMC) of INS children, including 28 in relapse, 23 in remission and 40 controls. No child had any sign of infection, but a higher Epstein\u2013Barr virus viral load was measured in the PBMC of relapsing patients. TLR-3 expression was increased in B cells only during INS remission. There was a negative correlation between proteinuria and TLR-3 expression in total and the main subsets of PBMC from INS patients. The expression of TLR-8 was also increased in both CD4+ T cells and B cells in INS remission. There was a negative correlation between proteinuria and TLR-8 expression in total PBMC, CD4+ T cells and B cells of INS patients. Nevertheless, TLR-3 and TLR-8 expression was normalized in all PBMC subsets in an additional group of 15 INS patients in remission with B cell repletion after rituximab therapy. Paradoxically, interferon (IFN) regulatory factor 3 transactivation was increased in PBMC of all INS patients. In-vitro secretion of IFN-\u03b1 and interleukin 6 were increased spontaneously in PBMC of INS remission patients, whereas PBMC from all INS patients displayed an impaired IFN-\u03b1 secretion after TLR-3 stimulation. Thus, TLR-3 pathway dysfunctions may be closely involved in INS pathogenesis.<\/jats:p>","DOI":"10.1111\/cei.12659","type":"journal-article","created":{"date-parts":[[2015,6,30]],"date-time":"2015-06-30T08:39:08Z","timestamp":1435653548000},"page":"332-345","source":"Crossref","is-referenced-by-count":8,"title":["Toll-like receptor 3 expression and function in childhood idiopathic nephrotic syndrome"],"prefix":"10.1093","volume":"182","author":[{"given":"A","family":"Jamin","sequence":"first","affiliation":[{"name":"INSERM U1149, CNRS ERL8252, Center for Research on Inflammation, Bichat Medical School, Paris, France"},{"name":"Paris Diderot, Sorbonne Paris Cit\u00e9 University, Inflamex Laboratory of Excellence, Paris, France"},{"name":"DHU Fire, Paris, France"},{"name":"Department of Pediatric Nephrology, Robert Debr\u00e9 Hospital, Assistance Publique-H\u00f4pitaux de Paris, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L","family":"Dehoux","sequence":"additional","affiliation":[{"name":"INSERM U1149, CNRS ERL8252, Center for Research on Inflammation, Bichat Medical School, Paris, France"},{"name":"Paris Diderot, Sorbonne Paris Cit\u00e9 University, Inflamex Laboratory of Excellence, Paris, France"},{"name":"DHU Fire, Paris, France"},{"name":"Department of Pediatric Nephrology, Robert Debr\u00e9 Hospital, Assistance Publique-H\u00f4pitaux de Paris, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C","family":"Dossier","sequence":"additional","affiliation":[{"name":"DHU Fire, Paris, France"},{"name":"Department of Pediatric Nephrology, Robert Debr\u00e9 Hospital, Assistance Publique-H\u00f4pitaux de Paris, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M","family":"Fila","sequence":"additional","affiliation":[{"name":"DHU Fire, Paris, France"},{"name":"Department of Pediatric Nephrology, Robert Debr\u00e9 Hospital, Assistance Publique-H\u00f4pitaux de Paris, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N","family":"Heming","sequence":"additional","affiliation":[{"name":"INSERM U1149, CNRS ERL8252, Center for Research on Inflammation, Bichat Medical School, Paris, France"},{"name":"Paris Diderot, Sorbonne Paris Cit\u00e9 University, Inflamex Laboratory of Excellence, Paris, France"},{"name":"DHU Fire, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R C","family":"Monteiro","sequence":"additional","affiliation":[{"name":"INSERM U1149, CNRS ERL8252, Center for Research on Inflammation, Bichat Medical School, Paris, France"},{"name":"Paris Diderot, Sorbonne Paris Cit\u00e9 University, Inflamex Laboratory of Excellence, Paris, France"},{"name":"DHU Fire, Paris, France"},{"name":"Immunology Laboratory, Xavier Bichat Hospital, Assistance Publique-H\u00f4pitaux de Paris, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G","family":"Desch\u00eanes","sequence":"additional","affiliation":[{"name":"INSERM U1149, CNRS ERL8252, Center for Research on Inflammation, Bichat Medical School, Paris, France"},{"name":"Paris Diderot, Sorbonne Paris Cit\u00e9 University, Inflamex Laboratory of Excellence, Paris, France"},{"name":"DHU Fire, Paris, France"},{"name":"Department of Pediatric Nephrology, Robert Debr\u00e9 Hospital, Assistance Publique-H\u00f4pitaux de Paris, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2015,9,30]]},"reference":[{"key":"2021122010175404100_cei12659-bib-0001","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1016\/S0140-6736(03)14184-0","article-title":"Nephrotic syndrome in 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