Extended Data Fig. 2: TDP-43 Peripheral pathology in SOD1 mouse models.
From: Muscle-derived miR-126 regulates TDP-43 axonal local synthesis and NMJ integrity in ALS models
a) Western blots of sciatic nerve axoplasms from 3 p120 B6SJL.SOD1G93A and 3 littermates for TDP-43 (43kD), pTDP-43 (43kD) and hSOD1 (17kD). Tubulin (55kD) was used as a loading control. A distinct 25kD additional band was observed in the TDP-43 blot of SOD1G93A samples, suggested to be C-terminal fragments, as previously reported10. b) Quantitative analysis of the pTDP-43/tubulin intensities within blots from sciatic nerve axoplasms of p120 B6SJL.SOD1G93A and littermates. n = 3 mice in each condition. Two-tailed unpaired student’s t-test, p = 0.5146. c) Representative western blot image and quantitative analysis of the TDP-43/tubulin intensities within blots from sciatic nerve axoplasms of p110 B6.SOD1G93A and littermates. n = 3 mice in each condition. TDP-43 (43kD), Tubulin (55kD). Two-tailed unpaired student’s t-test. n = 0.0582. d) Western blots of sciatic nerve axoplasms from 3 p357 SOD1G37R and 3 littermates for TDP-43 (43kD), pTDP-43 (43kD) and hSOD1 (17kD). Tubulin (55kD) was used as a loading control. A distinct 25kD additional band was observed in the TDP-43 blot of SOD1G37R samples, suggested to be C-terminal fragments as previously reported. e, f) Quantitative analysis of TDP-43/tubulin (e) and pTDP-43/tubulin (f) in sciatic nerve axoplasms of p357 SOD1G37R and littermates. n = 3 mice in each condition. Two-tailed unpaired student’s t-test, **p = 0.0081, *p < 0.0118. g) Western blots of gastrocnemius muscle lysates from 3 p120 B6SJL.SOD1G93A and 3 littermates for TDP-43 (43kD), pTDP-43 (43kD) and hSOD1 (17kD). Tubulin (55kD) was used as a loading control. h, i) Quantitative analysis of TDP-43/tubulin (h) and pTDP-43/tubulin (i) background subtracted thus the negative values in gastrocnemius muscles of B6SJL.SOD1G93A and littermates. n = 3 mice in each condition. Two-tailed unpaired student’s t-test, p = 0.1348(h), *p = 0.0355(i). j, k) Representative images and quantitative analyses of TDP-43/tubulin in gastrocnemius muscle lysates from p290 SOD1G37R (j) and P110 B6.SOD1G93A (k) mice versus their littermates. TDP-43 (43kD), Tubulin (55kD). n = 4 mice per condition (j); n = 3 mice per condition (k). Two-tailed, unpaired student’s t-test. **p = 0.0038(j), p = 0.7216(k).
