Table 1.
CFHR Gene Variations in aHUS Affect FHR and Factor H but not FHL1 Plasma Levels
| Variable | Intact FHL1 | Affected | Intact | |||||
|---|---|---|---|---|---|---|---|---|
| Factor H | FHR3 | FHR1 | FHR 4 | FHR2 | FHR5 | |||
| Factor HFHR3 | 100 | 50 | Factor HFHR3 | 50 | 100 | 100 | 100 | 100 |
| Factor HFHR3 | 100 | 50 | Factor HFHR3 | 50 | 100 | 100 | 100 | 100 |
| Factor HFHR1 | 100 | 50 | Factor HFHR1 | 100 | 50 | 100 | 100 | 100 |
| Factor HFHR1 | 100 | 50 | Factor HFHR1 | 100 | 50 | 100 | 100 | 100 |
| FHR1::Factor H | 100 | 100 | FHR1::Factor H | 150 | 100 | 100 | 100 | 100 |
| FHR1::Factor H | 100 | 100 | FHR1::Factor H | 150 | 100 | 100 | 100 | 100 |
| ΔΔFHR3FHR1 | 100 | 100 | 0 | 0 | 100 | 100 | 100 | |
| ΔFHR3FHR1 | 100 | 100 | 50 | 50 | 50 | 100 | 100 | |
| ΔΔFHR1FHR4 | 100 | 100 | 100 | 0 | 0 | 100 | 100 | |
Numerical data presented as % plasma levels.