Fig. 5
From: Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts
Analysis of TDP-43 sub-cellular localization in human fibroblasts and SKNBE cells. a Representative immunofluorescence images of TDP-43 (green) in primary fibroblasts from 4 healthy controls (CTRL) and 3 ALS patients carrying p.A382T mutation in TARDBP gene (mTDP-43), incubated with Mitotracker Red (red). Bar, 10 μm. b WB analysis of TDP-43 and TOMM20 proteins in total cell lysates (TOT) and mitochondria-enriched fractions (MT) of CTRL and mTDP-43 fibroblasts maintained in galactose medium for 48 h. p84 and tubulin (TUB) were used to test contamination with nuclear and cytoplasmic fractions, respectively. c WB analysis of TDP-43 and TOMM20 proteins in total cell lysates (TOT) and mitochondria-enriched fractions (MT) of SK-N-BE cells transfected for 48 h with flag-tagged wild-type (fTDP-43wt) and mutant p.M337V (fTDP-43p.M337V) TDP-43 constructs. Asterisk indicates flag-tagged TDP-43 proteins. p84 and tubulin (TUB) were used as nuclear and cytoplasmic markers, respectively. Different protein amounts were loaded for TOT (25 μg) and MT (15 μg) fractions