Figure 2

Residual IDUA activity in MPS I fibroblasts of patients with different phenotypes. IDUA activity for each patient is reported in Table 1. IDUA activity was measured in duplicate in each sample. All experiments were at least repeated once in independent cell cultures, the results of one representative experiment are shown. All values are mean ± standard deviation. Control range: 101–270 nmol × mg^-1 × hr^-1.