Mutations in the genes encoding the regulators or components of the complement system, such as Factor H (CFH), Factor H-related protein 5 (CFHR5), Factor I (CFI), membrane cofactor protein (CD46), thrombomodulin (THBD), or Factor B (CFB) and complement C3 protein (C3) are present in about 30% of C3 glomerulopathy patients [4–8], whereas acquired factors (autoantibodies) may be identified as well in a significant subgroup (40–80%)of these cases [9–11]. This evidence concerns the gene THBD and complement 3 glomerulopathy.