Fig. 1

Aberrant expression of Tweak and Fn14 in skeletal muscle of Smn^−/−;SMN2 SMA mice. a–g qPCR analysis of parvalbumin (a), Tweak (b), Fn14 (c), Pgc-1α (d), Mef2d (e), Glut-4 (f), and HKII (g) in triceps, gastrocnemius, TA, and quadriceps muscles from postnatal day (P) 0 (birth), P2 (pre-symptomatic), P5 (early symptomatic), P7 (late symptomatic), and P19 (end stage) Smn^−/−;SMN2 and wild-type (WT) mice. Normalized relative expressions are compared to WT P0. Data are mean ± SEM, n = 3-4 animals per experimental group, two-way ANOVA, Sidak’s multiple comparison test between genotypes, *p < 0.05, **p < 0.01, ***p < 0.001, ****p < 0.0001. h–i Quantification of NF-κB p50/actin protein levels in the TA of pre-symptomatic (P2) (h) and late-symptomatic (P7) (i) Smn^−/−;SMN2 mice and age-matched WT animals. Images are representative immunoblots. Data are mean ± SEM, n = 3–4 animals per experimental group, unpaired t-test, ns, not significant (h), p = 0.0215 (i). j Quantification of NF-κB p50/actin and p105/actin protein levels in the quadriceps (quad) of late-symptomatic (P7) Smn^−/−;SMN2 mice and age-matched WT animals. Images are representative immunoblots. Data are mean ± SEM, n = 3–4 animals per experimental group, two-way ANOVA, uncorrected Fisher’s LSD, ****p < 0.0001, ns, not significant. k qPCR analysis NF-κB-inducing kinase (NIK) in TA muscle of late-symptomatic P7 Smn^−/−;SMN2 and age-matched WT animals. Data are mean ± SEM, n = 3–4 animals per experimental group, unpaired t-test, p = 0.0094