{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T13:38:21Z","timestamp":1781876301768,"version":"3.54.5"},"reference-count":21,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2008,11,7]],"date-time":"2008-11-07T00:00:00Z","timestamp":1226016000000},"content-version":"vor","delay-in-days":6,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Immunol"],"published-print":{"date-parts":[[2008,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Griscelli syndrome type 2 is caused by mutations in the RAB27A gene and is a rare and potentially fatal immune disorder associated with hemophagocytic lymphohistiocytosis (HLH). Animal models could provide assistance for better understanding the mechanisms and finding new treatments. Rab27a\u2010deficient (<jats:italic>ashen<\/jats:italic>) mice do not spontaneously develop HLH. When injected with lymphocytic choriomeningitis virus (LCMV) strain WE, Rab27a\u2010deficient C57BL\/6 mice developed wasting disease, hypothermia, splenomegaly, cytopenia (anemia, neutropenia and thrombocytopenia), hypertriglyceridemia and increased levels of IFN\u2010\u03b3, TNF\u2010\u03b1, GM\u2010CSF, IL\u201012, CCL5 and IL\u201010. Activated macrophages with hemophagocytosis were found in liver sections of these mice. Compared with perforin\u2010deficient mice, LCMV\u2010infected Rab27a\u2010deficient mice showed a substantially better survival rate and slightly higher viral doses were needed to trigger HLH in Rab27a\u2010deficient mice. This study demonstrates that LCMV\u2010infected Rab27a\u2010deficient C57BL\/6 mice develop features consistent with HLH and, therefore, represent a murine model of HLH in human Griscelli syndrome type 2.<\/jats:p>","DOI":"10.1002\/eji.200838488","type":"journal-article","created":{"date-parts":[[2008,11,7]],"date-time":"2008-11-07T09:30:05Z","timestamp":1226050205000},"page":"3219-3225","source":"Crossref","is-referenced-by-count":59,"title":["A Griscelli syndrome type 2 murine model of hemophagocytic lymphohistiocytosis (HLH)"],"prefix":"10.1002","volume":"38","author":[{"given":"Jana","family":"Pachlopnik Schmid","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chen\u2010Hsuan","family":"Ho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julien","family":"Diana","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00e9rard","family":"Pivert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agn\u00e8s","family":"Lehuen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fr\u00e9d\u00e9ric","family":"Geissmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alain","family":"Fischer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Genevi\u00e8ve","family":"de Saint Basile","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2008,11,7]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(78)90858-6"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1038\/76024"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.140212797"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.0105-2896.2005.00224.x"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V78.11.2918.2918"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V89.11.4100"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2249.2003.02237.x"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-05-1980"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21039"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.176.8.4530"},{"key":"e_1_2_6_12_2","first-page":"299","article-title":"Haemophagocytic syndromes in adults: current concepts and challenges ahead","volume":"135","author":"Emmenegger U.","year":"2005","journal-title":"Swiss Med. 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