Figure 2

Ceruloplasmin activity and iron chelation treatment. (A) Effect of the iron chelator DFP on CSF levels of CP-ferroxidase activity. The patients in the ES group (n = 11 with two lumbar punctures: at baseline and at 6 months) displayed significantly higher CSF levels of CP activity than the patients in the DS group (F_(1,16)=13; p=0.002 (B) Effect of the iron chelator DFP on serum levels of CP-ferroxidase activity. The patients in the ES group showed significantly higher serum levels of CP activity than the patients in the DS group at 6 months (F_(1,35)=26; p=0.0001) and 12 months (F_(1,34)=5.2; p=0.028) but not at 18 months. (C) Effect of the iron chelator DFP on CSF levels of CP-ferroxidase activity as a function of D544E genotype. The DFP-treated patients with an AT genotype (n = 5) displayed significantly higher CSF levels of CP activity than the DFP-treated patients with an AA genotype (n = 6) (F_(1,8)=7; p=0.02). (D) Effect of the iron chelator DFP on serum levels of CP-ferroxidase activity as a function of D544E genotype. The DFP-treated patients with an AT genotype (AT; n = 5) displayed significantly higher levels of CP activity than DFP-treated patients with an AA genotype (AA; n = 15) at 6 months (F_(1,17)=7; p=0.02) but not at 12 or 18 months. (E) Correlation between CSF levels of CP-ferroxidase activity and the R2* MRI value in the SN. The change in CSF levels of CP activity between the baseline and the visit at 12 months were significantly correlated with the change in the SN’s R2* value (r=0.784; p=0.001). White circles represent the DFP-treated patients with an AA genotype. Grey circles represent the DFP-treated patients with an AA genotype with an AT genotype; the latter displayed higher levels of CP activity and a greater reduction in R2* in the SN.