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. Author manuscript; available in PMC: 2024 Aug 1.
Published in final edited form as: J Med Chem. 2019 Jun 25;62(13):6175–6189. doi: 10.1021/acs.jmedchem.9b00428

Figure 6. Effects of pharmacological chaperone compounds on GALNS enzyme activity.

Figure 6.

A) Effect of ezetimibe and pranlukast on GALNS activity in MPS IVA patient fibroblasts. The fibroblasts were treated with different concentrations of ezetimibe and pranlukast for 48 h after which the GALNS activity was measured in the cell lysate. Dotted line corresponds to WT activity levels. All assays were performed in triplicate (ANOVA Sidak t test * p < 0.05, ** p < 0.01, *** p < 0.001, **** p < 0.0001). B) Effect of ezetimibe (Eze) and pranlukast (Pran) on GALNS protein levels as determined by Western-blot analysis. The fibroblasts were treated with different concentrations of ezetimibe and pranlukast for 48 hrs after which the protein lysates from the fibroblasts were analyzed. The densities of specific bands were calculated by using NIH Image J 1.8.0. GALNS protein levels were normalized against β-actin density and reported as fold-change to WT levels (dotted line). All assays were performed in triplicate.