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. Author manuscript; available in PMC: 2009 Sep 5.
Published in final edited form as: Cell. 2008 Sep 5;134(5):769–781. doi: 10.1016/j.cell.2008.06.037

Figure 6.

Figure 6

PCs and PRs exhibit synergy in Gaucher and Tay-Sachs fibroblasts. Enzyme activities within patient-derived fibroblasts treated with a PC and a PR. In all the 3D plots (A–D, and F), PR and PC media concentrations are shown on the x and y-axes, and the mutant enzyme activities on the z-axis with the dosing schematic depicted at the bottom. Reported activities were normalized to the activity of untreated cells of the same type (left z axis) and expressed as the percentage of WT enzyme activity (right z axis). αG269S/1278insTATC HexA fibroblasts were exposed to MG-132 (E) and the Hex α-site activities were measured as a function of time. The data in (E) were reported as mean ± SEM.