A new reactive-centre P1 residue mutation (444Arg~Ser), has been identified in a dysfunctional CI inhibitor protein, C1 inhibitor(Ba), contained in a type II hereditary angioedema plasma. This substitution is compatible with a point mutation of the 444Arg codon (CGC~AGC), and represents the first non-histidine, non-cysteine P 1 residue mutant described for C 1 inhibitor.