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Mice with deficiency of Limp\u20102 (the murine homologue) develop tubular proteinuria but not focal and segmental glomerulosclerosis and they have a defect in macrophage function. To further elucidate the role of Limp\u20102 in immune function, we induced anti\u2010glomerular basement membrane (GBM) model of crescentic glomerulonephritis in wild\u2010type (WT) and Limp\u20102<jats:sup>\u2212\/\u2212<\/jats:sup> littermates by intraperitoneal injections of nephrotoxic sheep serum. Renal injury and immune responses were assessed on day 14. Compared with WT, Limp\u20102<jats:sup>\u2212\/\u2212<\/jats:sup> mice had significantly reduced crescent formation, interstitial inflammation and a trend to reduced tubulointerstitial injury. On day 1 during the heterologous phase of the disease, albuminuria was significantly increased in WT but not in Limp\u20102<jats:sup>\u2212\/\u2212<\/jats:sup> mice. On day 14, albuminuria and renal function were similar in the two groups. There was, however, a significant reduction in the influx of glomerular macrophages and CD4<jats:sup>+<\/jats:sup> T cells in Limp\u20102<jats:sup>\u2212\/\u2212<\/jats:sup> mice. Interleukin (IL)\u20104 and macrophage chemoattractant protein\u20101 (MCP\u20101) mRNA expression levels were significantly reduced. Despite the reduction in numbers of infiltrating cells, flow cytometry showed no difference in macrophage or T\u2010cell numbers in the peripheral blood from untreated mice. The systemic humoral immune response, determined by glomerular mouse immunoglobulin G (IgG) deposition and mouse anti\u2010sheep IgG subclass production, was similar in both groups. These data suggest that absence of Limp\u20102 reduces inflammation in experimental crescentic glomerulonephritis with decreased macrophage and T\u2010cell infiltration in the kidney. It suggests an important role for Limp\u20102 in mediating the inflammatory response.<\/jats:p>","DOI":"10.1038\/icb.2013.104","type":"journal-article","created":{"date-parts":[[2014,1,7]],"date-time":"2014-01-07T10:58:23Z","timestamp":1389092303000},"page":"400-408","source":"Crossref","is-referenced-by-count":6,"title":["Absence of the lysosomal protein Limp\u20102 attenuates renal injury in crescentic glomerulonephritis"],"prefix":"10.1111","volume":"92","author":[{"given":"Darren Hiu Kwong","family":"Lee","sequence":"first","affiliation":[{"name":"Department of Nephrology, Austin Health Melbourne Victoria Australia"},{"name":"Department of Medicine, Austin Health, University of Melbourne Melbourne Victoria Australia"},{"name":"Department of Renal Medicine, Faculty of Medicine, Nursing and Health Sciences, Eastern Health Clinical School, Monash University Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Poh\u2010Yi","family":"Gan","sequence":"additional","affiliation":[{"name":"Department of Medicine, Monash Medical Centre, Centre of Inflammatory Diseases, Monash University Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marina","family":"Katerelos","sequence":"additional","affiliation":[{"name":"The Institute for Breathing and Sleep, Austin Health Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Scott Andrew","family":"Fraser","sequence":"additional","affiliation":[{"name":"The Institute for Breathing and Sleep, Austin Health Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kurt","family":"Gleich","sequence":"additional","affiliation":[{"name":"The Institute for Breathing and Sleep, Austin Health Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephen Roger","family":"Holdsworth","sequence":"additional","affiliation":[{"name":"Department of Medicine, Monash Medical Centre, Centre of Inflammatory Diseases, Monash University Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David Anthony","family":"Power","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Austin Health Melbourne Victoria Australia"},{"name":"Department of Medicine, Austin Health, University of Melbourne Melbourne Victoria Australia"},{"name":"The Institute for Breathing and Sleep, Austin Health Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,1,7]]},"reference":[{"key":"e_1_2_7_1_1","first-page":"87","article-title":"Action myoclonus\u2010renal failure syndrome: a previously unrecognized neurological disorder unmasked by advances in nephrology","volume":"43","author":"Andermann E","year":"1986","journal-title":"Adv Neurol"},{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh263"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2007.12.019"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000238424.23177.5e"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajprenal.00015.2011"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-009-1381-9"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M110.146761"},{"key":"e_1_2_7_8_1","first-page":"10","article-title":"The participation of macrophages, glomerular procoagulant activity, and factor VIII in glomerular fibrin deposition. 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