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. 2016 Feb 10;36(6):1858–1870. doi: 10.1523/JNEUROSCI.3095-15.2016

Figure 8.

Figure 8.

4-PBA enhances GALC levels and activity. A, GALC levels were measured from total lysates of transfected cells expressing WT or mutant GALC in combination with various cis-polymorphisms after treatment with 2 mm 4-PBA for 24 h. The chaperone increased the 80 kDa GALC precursor in WT and in all mutants, but not relative levels of processed GALC fragments. B, GALC activities were measured from transfected cells expressing the WT and G286D GALCs with or without treatment with 2 mm of 4-PBA. 4-PBA significantly enhanced the GALC activities in cells expressing WT, G286D, and T529M GALCs. *p < 0.05 (Student's t test). **p < 0.01 (Student's t test). ***p < 0.001 (Student's t test). n.s., Not significant. C, The addition of 4-PBA for 24 h increased the trafficking to the lysosome (LAMP2; green) of WT and all mutant GALCs (red) in transfected HEK-293T cells, possibly due to the overall increase in GALC precursor by 4-PBA. Orthogonal views (XY, XZ, and YZ) of a z-stack. White lines indicate section positions. Scale bar: C, 5 μm.