1. Identification of Z-FA-FMK as a potent compound that increases SMN protein expression in HEK293 and patient fibroblast cells.
Identification of Z-FA-FMK as a potent compound that increases SMN protein expression in HEK293 and patient fibroblast cells. (A) Fluorescent images showed the increased GFP fluorescence at 2 d after treatment with different concentrations of Z-FA-FMK. Scale bar, 50 μm. (B, C) Western blot analysis of these samples revealed a dose-dependent increase in the expression of SMNΔ7-GFP proteins after the treatment of Z-FA-FMK. (D) qRT-PCR showing the mRNA expression in SMN2-GFP cells after treatment with different concentrations of Z-FA-FMK. n = 3. (E–G) In SMA type I fibroblast cells, vehicle (DMSO) or Z-FA-FMK were applied for 2 d, followed by analysis of the protein (E, F) and mRNA (G) expression in these cells. n = 3. Data were presented as mean ± SEM, *P < 0.05, as compared with the DMSO-treated group by two-tailed t test.
Yiran Wang et al. LSA 2019;2:e
© 2019 Wang et al.