Table 1.
Representative abnormalities in complement leading to renal disease
| Components/related molecules | Diseases |
| Complement C3 | C3 glomerulopathy (DDD), aHUS |
| Factor H | C3 glomerulopathy (DDD/C3GN), aHUS |
| Factor I | C3 glomerulopathy (C3GN), aHUS |
| MCP | aHUS |
| Factor B | aHUS |
| CFHR5 | Familial C3 glomerulopathy (CFHR5 nepropathy) |
| CFHR3-1 | Familial C3 glomerulopathy |
| CFHR1/3 | IgA nephropathy, aHUS |
| Factor B autoantibody | C3 glomerulopathy (DDD) |
| Factor H autoantibody | C3 glomerulopathy (DDD/C3GN) |
| Bb (activated factor B) | HUS, ANCA-associated vasculitis |
| C3Nef | C3 glomerulopathy (DDD, C3GN) |
| Soluble C5b-9 | HUS, TTP, ANCA-associated vasculitis |
| C3a | ANCA-associated vasculitis, TTP |
| C5a | ANCA-associated vasculitis |
| C1q/C1qR | C1q nephropathy |
| Properdin | TI injury due to massive proteinuria |
| C5 | ANCA-associated vasculitis |
| Factor B | ANCA-associated vasculitis |
| CRaR | TI inflammation, IRI |
| C5aR | IRI |
| Factor H | IRI |
| C5b-9 | IRI |
| CD59 | IRI |
DDD: Dense deposit disease; aHUS: Atypical hemolytic uremic syndrome; C3GN: C3 glomerulonephritis; MCP: Membrane co-factor protein; CFHR5: Complement factor H-related protein; ANCA: Anti neutrophil cytoplasmic antibody; C3Nef: C3 nephritic factor; TTP: Thrombotic thrombocytopenic purpura; TI: Tubulo-interstitial; IRI: Ischemia-reperfusion-injury.