Fig. 8
HDAC inhibition in LSD. a RT-qPCR of NEU1 in SAHA- or DMSO-treated sialidosis fibroblasts, (SAHA 20 μM for 24 h; n = 8 biologically independent samples). Type I = attenuated form of sialidosis; Type II = severe form of sialidosis. b Top, representative immunoblot of fibroblasts isolated from two patients with sialidosis type I and two patients with sialidosis type II were treated with SAHA (S) (20 μM for 24 h) or DMSO (D) and probed with anti-NEU1 antibody; bottom, Coomassie stained immunoblot used as loading control. c NEU1 activity in fibroblasts isolated from two patients with sialidosis type I and two patients with sialidosis type II treated with SAHA (S)- or DMSO (D)-treated (SAHA, 20 μM for 24 h; n = 7 biologically independent samples). d ChIP analysis of the promoter of NEU1 performed with acetyl histone H3 Lys 14 antibody (Acetyl-H3K14) in fibroblasts from one type I sialidosis fibroblasts treated with SAHA (20 μM for 24 h) or DMSO (n = 3 biologically independent samples). Boxes represent the mean value and bar inside the box represents median value; upper bar represents maximum of distribution; lower bar represents minimum of distribution (95% confidence level). Graphs are presented as mean ± SD. Statistical analysis was performed using the Student t-test. *p < 0.05, ***p < 0.001, ****p < 0.0001
