Abstract
Polymorphism of serum Factor B (Bf) of the alternative complement (C) pathway was determined in 61 (non-Black) children with steroid-responsive INS ages 1 to 15 and in 70 (non-Black, unmatched) normals using prolonged agarose electrophoresis with immunofixation. Immunochemical assays of C3 and Factor B and functional assays of the alternative (APH50) C pathways and Factor D, were also performed.
Bf gene frequencies were significantly different between INS and normals with an increase in BfF and a decrease in BfS (INS: BfS-.7213, BfF-.2623, BfF1-.0164; Normals: BfS-.8500, BfF-.1357, BfF1-.0143; ×2=5.944 with Yates; correction, p< .05). Phenotype frequencies (excluding rare variants) were not different (INS: BfS-35, BfFS+BfF-24; Normals: BfS-50, BfFS+BfF=18; ×2=2.2747 with Yates' correction, p>.10). BfF1 was similar between groups and not present in 2 patients with INS and diabetes mellites. C3 type was not different between INS and normals.
APH50 was reduced during relapse with INS and had an overall correlation with serum Factor B (r=.3818, p<.01), albumin (r=.4295, p< .01) and C3 (r=.3687, p<.01). Factor B in INS also correlated with serum albumin (r=.5938, p< .001). Factor D showed no correlation with APH50, Factor B or albumin. We conclude that the BfF gene is increased in INS and that alternative C pathway function is reduced in relapsing INS.
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McLean, R., Kennedy, T., Ballow, M. et al. INCREASED FREQUENCY OF THE BfF GENE IN THE IDIOPATHIC NEPHROTIC SYNDROME (INS): STUDIES OF THE ALTERNATIVE COMPLEMENT PATHWAY IN INS. Pediatr Res 14, 1012 (1980). https://doi.org/10.1203/00006450-198008000-00233
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DOI: https://doi.org/10.1203/00006450-198008000-00233
