Fig. 2.

SGPL1 deficiency leads to accumulation of upstream sphingolipids in patient dermal fibroblasts and the isogenic SGPL1 knockout HeLa cell line. A. FIB_WT (control) showed a detectable band at 60 KDa while both patient cell lines were SGPL1 null. B. Similarly, S1P Lyase was expressed in HeLa control lysates and absent in knockout cells. C, D. Levels of sphingolipid intermediates in control and patient-derived dermal fibroblasts as well as isogenic HeLa cell lines. E. S1P lyase deficiency alters the sphingolipid rheostat leading to accumulation of upstream lipids. (*p < 0.05, **p < 0.01, ***p < 0.001, ****p.0001) Data are presented as the mean of three experiments.