Aberrant galactosylation of IgA1 is involved in the genetic susceptibility of Chinese patients with IgA nephropathy.

BACKGROUND Immunoglobulin A nephropathy (IgAN) is associated with genetic and environmental factors, and undergalactosylation of IgA1 in the serum is considered to be a contributor to pathogenesis of IgAN. The present study was conducted to detect the galactose- (Gal) deficient IgA1 level in Chinese IgAN patients and their family members. METHODS Sixty-three IgAN patients were enrolled, where 32 first-degree relatives of 19 patients and 44 spouses of 44 patients were recruited. Healthy blood donors (n = 39) were used as normal controls. Biotinylated HAA (Helix aspersa) was utilized to detect the Gal-deficient IgA1 in enzyme-linked immunosorbent assay (ELISA). All the results were corrected by serum IgA1 concentration. RESULTS Compared with normal controls, the sera IgA1 of patients and their first-degree relatives demonstrated increased Gal-deficient IgAl level (0.17 +/- 0.09 versus 0.10 +/- 0.04, P = 0.001; 0.14 +/- 0.07 versus 0.10 +/- 0.04, P = 0.028); no significant difference between patients and their first-degree relatives was detected (0.17 +/- 0.09 versus 0.14 +/- 0.07, P = 0.127). In contrast, serum Gal-deficient IgA1 level of IgAN patients was higher than their counterpart spouses and normal controls (0.18 +/- 0.13 versus 0.14 +/- 0.09, P = 0.009; 0.18 +/- 0.13 versus 0.10 +/- 0.04, P = 0.001), while that of patients' spouses was comparable with normal controls (0.14 +/- 0.09 versus 0.10 +/- 0.04, P = 0.075). There was no correlation between clinicopathological data and serum Gal-deficient IgA1 level. CONCLUSION The patients with IgAN and their first relatives showed significant higher Gal-deficient IgA1 level than healthy controls, whereas patients' spouses were the same as healthy controls. It can be suggested that the Gal-deficient IgA1 might be inherited in Chinese patients with IgAN.