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. Author manuscript; available in PMC: 2012 Nov 1.
Published in final edited form as: Mol Genet Metab. 2011 Nov 1;104(3):338–345. doi: 10.1016/j.ymgme.2011.05.021

Figure 3. Reduced thioester load in PTC124-treated lymphoblast cells.

Figure 3

Lymphoblasts isolated from three different patients carrying PPT1 nonsense mutations were pretreated with PTC124 (5 µg/ml) for 48 h and then labeled with [35S] cysteine for 6h. Labeled thioesters were extracted and resolved by high performance thin layer chromatography. Arrows indicate thioester bands that are reduced in intensity following PTC124 (5 µg/ml)-treatment of the cells. Lower panel shows densitometric analysis of the thioester bands in PTC124-treated cells with respect to untreated control.