Fig. 2
From: Proteasome impairment in neural cells derived from HMSN-P patient iPSCs
Cellular phenotypes in HMSN-P patient iPS-MNs. a Immunostaining for TFG in purified MNs by HB9::GFP sorting. Scale bar = 10 μm. b Quantification of area of TFG-positive puncta in purified MNs by HB9::GFP sorting measured by high-content analysis (n = 3, *p < 0.05 by Student t-test). Error bars are ± s.e.m. c Immunoblot analysis of TFG in control and HMSN-P patient iPS-MNs. β-actin was used as loading control. d Quantification of the TFG protein levels in control and HMSN-P patient iPS-MNs (n = 3, *p < 0.05 by Student t-test). e Proteasome activity of control and HMSN-P patient iPS-MNs (n = 3, *p < 0.05 by Student t-test). Error bars are ± s.e.m. f Immunoblot analysis of ubiquitinated proteins in control and HMSN-P patient iPS-MNs. β-actin was used as loading control. g Quantification of the level of HMW ubiquitinated proteins in control and HMSN-P patient iPS-MNs (n = 3, *p < 0.05 by Student t-test). Error bars are ± s.e.m. h Survival of control and HMSN-P patient iPS-MNs after MG132 exposure (n = 3, ** p < 0.01, by Student t-test). i Immunoblot analysis of cleaved caspase-3 in control and HMSN-P patient iPS-MNs exposed to vehicle or MG132. β-actin was used as loading control. DMSO: dimethyl sulfoxide