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. 2019 May 8;7(7):e00733. doi: 10.1002/mgg3.733

Figure 2.

Figure 2

S1P Pro1003Ser has enhanced activity and accumulates in the Golgi. mRNA expression levels in cultured control‐ and patient‐derived skin fibroblasts of (a) SREBP1 and 2 target genes after 4 hr of treatment with the indicated concentrations of mevastatin and (b) UPR and ATF6 target genes following treatment with 1 µg/ml of tunicamycin for 4, 8, and 12 hr. (c) SRD‐12B cells transiently transfected with FAS‐Luc, Renilla, and WT S1P or S1P Pro1003Ser plasmids as indicated, after 10 hr, cells were treated with DMEM/F12 for 16 hr. Luciferase activities were measured and the ratio between firefly and Renilla luciferase activities was determined. (d) Immunofluorescence of S1P, ER marker KDEL, and Golgi marker GM130 in S1P Pro1003Ser patient and control fibroblasts. Cells were transfected with FLAG‐tagged WT S1P or S1P Pro1003Ser as indicated. Representative images are shown. All data are expressed as mean S.E. of three to five independent experiments, *p < 0.05