Table 1 Restitution of Classical (CP), Lectin (LP) and Alternative pathway (AP) activation in serum from 6 C2-deficient patients by addition of rhC2

Restituted serum^a Patient no.	CP	LP^c	AP
1	+++^b	±	+++
2	+++	-	+++
3	+++	±	+++
4	+++	+	+++
5	+++	++	+++
6	+++	-	+++

^arhC2 was added to C2-deficient serum, final concentration 25 μg/ml.
^bComplement activation tested by ELISA. +++ = 100% activation, ++ = 50%, + = 20%, - = no activation.
^cLP activity reflects the MBL concentration governed by MBL genotype. Patient 5 is homozygous for the wild-type structural gene variant (genotype A/A), while the other patients are heterozygous for this allele.

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