Fig. 4.

a Antibody titers are correlated with the lysoGb3 plasma levels (Spearman r = 0.63, p < 0.0001) and inversely correlated with the residual enzymatic activity (RRA% × 100) of agalsidase in sera (Spearman r = − 0.74, p < 0.0001). b IgG1–4 subclasses levels (μg/ml) depending on total IgG status (Tot-IgG) and inhibition (inhib+ corresponding to RRA < 0,74), Mann-Whitney test (*: p < 0,05; **: p < 0,01; ***: p < 0,001). c Alpha-galactosidase A activity is lower in leucocytes cultured with Ab-positive compared to Ab-negative serum (Mann-Whitney test, median: 42.0 (n = 7) vs 75.6 (n = 9), p = 0.04). d Anti-agalsidase antibodies have no effect on alpha-galactosidase B (NAGA): there is no difference in NAGA enzyme activity when incubated with Ab-positive or Ab-negative serum (n = 12 and 11 respectively, unit in pmol/min/μg; Mann-Whitney test, p = 0.44)