Figure 3.

Functional evaluation of rGal variants after drug-induced translational readthrough.

(A) Activity of full-length rGal variants, produced by readthrough induction, towards the 4-MU-α-D-galactopyranoside fluorogenic substrate. Results, indicated as % of wild-type rGal, are reported as mean ± standard deviation from three independent experiments. (B) Specific activity, referred as the ratio between enzyme activity (panel A) and protein amount (Fig. 2B), of rGal nonsense variants after induction of translational readthrough. The most responsive rGal variants in terms of specific activity (Q119X and Q321X) or protein levels (C56X and W209X) were selected (striped bars) for further characterization. nd, not detectable.