Cellular and biochemical response to chaperone versus substrate reduction therapies in neuropathic Gaucher disease
Fig 5
Assessment of cell viability and metabolic status in fibroblasts.
A. Control (WT) fibroblasts were treated with 1, 10 or 100 μM of AMB and 0.1, 1 or 10 μM of EGT for 5 days and were submitted to the CCK-8 cell viability assay and ATP content. The obtain cell counting assay, CCK-8, and ATP results were normalized to the untreated cells. Additionally, the ratio ATP/CCK-8 (cell viability) was estimated. Values are expressed as average ±SEM, n = 3. * p<0.05 compared with an untreated group. B. Fibroblasts derived from three GD2 patients, P6 with L444P/L444P;RecΔ55;RecNCiI, P7 with L444P/L444P;R495P/R495P;A456P GBA variations, P9 with L444P/D409H, and one GD3 patient with L444P/L444P mutation, (P5) were treated with 1, 10, 100 μM of AMB and 0.1, 1,10 μM of EGT for 5 days. The CCK-8 cell viability assay and ATP content were analyzed. The obtained cell counting assay, CCK-8, and ATP results were normalized in relationship to the untreated cells. ATP/CCK-8 (cell viability) ratio was estimated. Values are expressed as average ±SEM, n = 4. * p<0.05 compared with an untreated group.