Figure 3.

TRIM37 gene mutation causes a defect in the import of peroxisomal matrix proteins. (A) Diagram of TRIM37 structure and the fs mutation at Arg166. Loss of TRIM37 protein in human patient fibroblasts (AG02122) was verified by Western blot. (B) Normal (AG21802) or patient fibroblast cells (AG02122) were stained with antibodies against PTS1 proteins and PMP70. (C) GFP-RFP-SKL or GFP-PECR constructs were transfected into the fibroblasts. The images were taken 24 h after transfection. The expression of GFP-RFP-SKL and GFP-PECR constructs in patient cells was very weak, and hence relatively higher exposure times were used to capture the signal compared with those for normal cells. Bars: 10 µm; (insets) 5 µm.