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. Author manuscript; available in PMC: 2016 Dec 27.
Published in final edited form as: Nat Med. 2016 Jun 27;22(8):869–878. doi: 10.1038/nm.4130

Figure 1. TDP-43 co-localizes with and accumulates in mitochondria in individuals with ALS and FTD.

Figure 1

(a,b) Representative images of TOM20 and TDP-43 in human motor neurons in lumbar spinal cords of sporadic ALS (n = 6) (a), or human cortical neurons in cortices of sporadic FTD (n = 4) (b). Control neurons are from age-matched normal individuals (n = 5 for spinal cords and 3 for cortices). Right panels show line-scan analysis (by Image J RGB Profile Plot plugin) along the solid white lines depicted in the merged images to the left. (c,d) Reconstructed three-dimension (3D) images of the neurons depicted in a and b, respectively. (e, f) Representative immunoblot and quantification (n = 3) of TDP-43 levels in mitochondria isolated from age matched control (n = 6) and sporadic ALS (n = 8) spinal cords (e), or age matched control (n = 6) and sporadic FTD (n = 7) cortices (f). (g,h) Representative immunoblot (n = 3) of TDP-43 in sub-mitochondrial fractions prepared from ALS spinal cords (g) and FTD cortices (h). (i) Immuno-EM of TDP-43 in mitochondria from sporadic ALS spinal cord or sporadic FTD cortex. Red arrowheads point to immunogold labeled TDP-43. The right panel shows quantification using thin EM sections with 50 nm thickness. n = 8, 6, 6 and 6 respectively. Data are means ± s.e.m of triplicate independent experiments throughout. Statistics: one-way analysis of variance (ANOVA) followed by Tukey’s multiple comparison test. *P < 0.05, **P < 0.01 and ***P < 0.001.