Fig. 7.
Role of the USP7–SMAD2 pathway in neurons and patient tissues. (A) Mouse neurons were transduced with SOD1G85R or SOD1WT HSVs and treated with vehicle (DMSO) or the USP7 inhibitor HBX41108. (Scale bar, 150 μm.) Live cells were loaded with the calcein AM dye to determine cell viability (Left). Quantification of the cell viability (Right; n = 8; n.s., nonsignificant; *P < 0.05). (B) Representative Western blotting of human spinal cord tissue lysates derived from ALS patients and controls show up-regulation of SMAD2 in the patients’ tissues (Left). Quantification of the SMAD2 protein levels (Right; n = 8 for control and n = 24 for ALS; **P < 0.01). Error bars indicate ± SEM.