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Loss of the Golgi matrix protein 130 cause aberrant IgA1 glycosylation in IgA nephropathy

American Journal of Nephrology Apr 01, 2019

Wang C, et al. - Researchers focused on the role of Golgi matrix protein 130 (GM130) in glycosylate deficiency IgA1 (Gd-IgA1) production, as aberrant O-glycosylation IgA1 production is a major factor in the pathogenesis of IgA nephropathy. Participants included 27 IgA nephropathy patients, 12 patients with chronic tonsillitis, 15 non-IgAN chronic kidney disease patients, and 15 healthy volunteers as healthy control. They used immunofluorescence staining and Western blotting to analyze the expression of GM130 in Tonsillar tissue. Via flow cytometry, they assessed the expression in peripheral blood mononuclear cells (PBMCs). In tonsil tissues and PBMC of IgAN patients, they detected significantly decreased expression of GM130; patients with IgAN had significantly increased Gd-IgA1 level and decreased C1GALT1 expression (β1,3-Gal transferase). Findings revealed a negative association of GM130 expression with Gd-IgA1 production. In IgAN patients, GM130 negatively regulated C1GALT1 expression and thereby, displayed a crucial role in IgA1 O-glycans deficiency in these patients.
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