Table 2.
Effects of genetic deficiency for complement components on lupus-like disease in murine models of lupus.
| Knockout genes | Classical pathway components | Alternative pathway (AP) components | LP and AP components | ||||||
|---|---|---|---|---|---|---|---|---|---|
| C1q | C4 | C3 | Factor B | Factor D | Mannose-binding lectin-associated serine protease-1/3 | ||||
| Strain | 129/Ora × C57BL/6 | MRL+/+ | B6, 129 × C57BL/6, BALB/c × 129 × C57BL/6 | (129/Sv × C57BL/6)lpr | (129/Sv × C57BL/6)lpr | MRL/lpr | MRL/lpr | MRL/lpr | MRL/lpr |
| H2 haplotype | H2b/b | H2k/k | H2b/b | H2b/b | H2b/b | H2k/k | H2b/b | H2k/k | H2k/k |
| Antinuclear antibody titer | Increased (55%) | Increased | Increased | No difference | |||||
| Serum anti-DNA levels | Increased | Increased | Increased | No difference | No difference | Decreased | No difference | No difference | |
| Serum C3 levels | Increased | Increased | Increased | ||||||
| Glomerular IgG deposits | Increased | Increased | No difference | Increased | Decreased | No difference | No difference | ||
| Glomerular C3 deposits | Decreased | Decreased | Decreased | ||||||
| Proteinuria | No difference | Increased | Decreased | No difference | Significantly decreased | ||||
| Glomerulo-nephritis (renal score) | Developed (25%) | Developed | No difference | Developed | No difference | No difference | Improved | Improved | Improved |
| Multiple apoptotic cell bodies and immune deposits | Hypercellularity, glomerular enlargement, mesangial thickening | ||||||||
| Survival | Decreased | Decreased | No change | Improved | No change | No change | |||
| Reference | (15) | (17) | (32) | (16) | (16) | (34) | (18) | (19) | This study |
The blue, green, yellow, or orange shades represent phenotypes of mice lacking complement component of the classical pathway, C3, the alternative pathway, or the alternative pathway and lectin pathway, respectively. Red or blue letters indicate that the corresponding complement component defects are pathogenic or protective against the development of murine lupus-like disease, respectively.
CP, classical pathway; AP, alternative pathway; LP, lectin pathway; MASP-1/3, mannose-binding lectin-associated serine protease-1/3; ANA, anti-nuclear antibody.