{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T14:38:00Z","timestamp":1790260680254,"version":"4.1.0"},"reference-count":28,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2005,9,26]],"date-time":"2005-09-26T00:00:00Z","timestamp":1127692800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Internal Medicine"],"published-print":{"date-parts":[[2005,11]]},"abstract":"<jats:title>Abstract.<\/jats:title><jats:p><jats:bold>Purpose.\u2002<\/jats:bold> Aberrant O\u2010glycosylation of serum IgA<jats:sub>1<\/jats:sub> is presumed to be one of the main pathogenesis of immunoglobulin A nephropathy (IgAN). <jats:italic>\u03b2<\/jats:italic>1,3\u2010galactosyltransferase (<jats:italic>\u03b2<\/jats:italic>1,3GT), whose activity requires coexistence of a specific chaperone, is the main enzyme which participate in the glycosylation process. The current study was carried out to elucidate the expression level of <jats:italic>\u03b2<\/jats:italic>1,3GT (C1GALT1) and its chaperone (Cosmc) in IgAN, and their relationships with clinical features as well as IgA glycosylation level.<\/jats:p><jats:p><jats:bold>Design, setting and subjects.\u2002<\/jats:bold> Forty\u2010one patients with IgAN, 21 patients with non\u2010IgAN glomerulonephritis and 26 normal controls were included in the present study. Peripheral B lymphocytes were isolated, and then expression level of C1GALT1 and Cosmc were quantitatively measured by real\u2010time reverse transcriptase polymerase chain reaction (RT\u2010PCR). Serum IgA level and glycosylation level were determined by enzyme\u2010linked immunosorbent assay (ELISA) and VV lectin\u2010binding method. Correlation analysis was performed between C1GALT1\/Cosmc expression levels and clinical manifestations (severe proteinuria, renal dysfunction, gross haematuria).<\/jats:p><jats:p><jats:bold>Results.\u2002<\/jats:bold> B\u2010lymphocyte Cosmc gene expression level was significantly lower in IgAN patients than that of normal control and non\u2010IgAN patients (<jats:italic>P<\/jats:italic>\u2003&lt;\u20030.05), whilst no apparent disparity was observed in C1GALT1 expression level. Cosmc expression showed a negative correlation with IgA O\u2010glycosylation level indicated by VV lectin\u2010binding assay. Statistical analysis also indicated that the level of Cosmc expression was negatively correlated with severe proteinuria (<jats:italic>P<\/jats:italic>\u2003&lt;\u20030.05) instead of gross haematuria (<jats:italic>P<\/jats:italic>\u2003&gt;\u20030.05).<\/jats:p><jats:p><jats:bold>Conclusion.\u2002<\/jats:bold> These data suggested that the aberrant IgA O\u2010glycosylation in IgAN was resulted from a downregulation of <jats:italic>\u03b2<\/jats:italic>1,3GT chaperone (Cosmc) expression in B lymphocyte, which is closely associated with clinical characteristics of the disease. This downregulation might be one of the fundamental pathogenic abnormalities in IgAN.<\/jats:p>","DOI":"10.1111\/j.1365-2796.2005.01558.x","type":"journal-article","created":{"date-parts":[[2005,9,26]],"date-time":"2005-09-26T14:36:03Z","timestamp":1127745363000},"page":"467-477","source":"Crossref","is-referenced-by-count":71,"title":["Peripheral B lymphocyte <i>\u03b2<\/i>1,3\u2010galactosyltransferase and chaperone expression in immunoglobulin A nephropathy"],"prefix":"10.1111","volume":"258","author":[{"given":"W.","family":"QIN","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Q.","family":"ZHOU","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.\u2010C.","family":"YANG","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Z.","family":"LI","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.\u2010H.","family":"SU","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"LUO","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.\u2010M.","family":"FAN","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2005,9,26]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.V11122395"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.V94577"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1523-1755.2001.060003969.x"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0272-6386(03)00743-1"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/12.4.701"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M109060200"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.262438199"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M205839200"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0272-6386(97)90472-8"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0272-6386(97)90456-X"},{"key":"e_1_2_7_12_2","doi-asserted-by":"publisher","DOI":"10.1097\/00004872-200311000-00002"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V88.1.222.222"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/13.4.930"},{"key":"e_1_2_7_15_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/29.9.e45"},{"key":"e_1_2_7_16_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00018-002-8489-4"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI116434"},{"key":"e_1_2_7_18_2","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.V9112048"},{"key":"e_1_2_7_19_2","first-page":"714","article-title":"Removal systems of immunoglobulin A and immunoglobulin A containing complexes in IgA nephropathy and cirrhosis patients. 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