Identification of pharmacological chaperones as potential therapeutic agents to treat phenylketonuria
J. Clin. Invest. Angel L. Pey, et al. 118:2858 doi:10.1172/JCI34355 [
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Figure 2Representative thermal denaturation profiles of WT-PAH as monitored by ANS fluorescence in the absence or presence of different concentrations of hit compounds I (
A), II (
B), III (
C), and IV (
D).
Experiments were performed using 0 (black), 25 (red), 50 (green), and 100 (light blue) μM of each compound in 2.5% DMSO. Plots are representative of at least 3 independent experiments. Insets show titrations of WT-PAH with compounds I–IV monitored by quenching of Trp-emission fluorescence spectroscopy.