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. 2018 May 4;31:226–242. doi: 10.1016/j.ebiom.2018.04.024

Fig. 1.

Fig. 1

Dysregulation of the GC-KLF15-BCAA pathway in severe SMA mice and human SMA patients. a. qPCR analysis of GRα mRNA in four different skeletal muscles (triceps brachii (triceps), gastrocnemius (gastro), tibialis anterior (TA) and quadriceps femoris (quad)) of post-natal day (P) 2 Smn−/−;SMN2 mice compared to WT animals. Data represent mean ± SD; n = 3–4 animals per group; two-tailed t-test; triceps: p = 0.0113; gastro: p = 0.0487; TA: p = 0.0176; quad: p = 0.0042. b. qPCR analysis of GRβ mRNA in four different skeletal muscles of P7 Smn−/−;SMN2 mice compared to WT animals. Data represent mean ± SD; n = 3–4 animals per group; two-tailed t-test; triceps: p = 0.0075; gastro: p = 0.004; TA: p = 0.0352; quad: p = 0.0008. c. qPCR analysis of Klf15 mRNA in four different skeletal muscles of Smn−/−;SMN2 mice compared to WT animals at P0, P2, P5, P7 and P10. Data represent mean ± SD; n = 3–4 animals per group; two-way ANOVA; ***p < 0.001, ****p < 0.0001. d. BCAA metabolism effector genes (mRNA) dysregulated in triceps of P2 and P7 Smn−/−;SMN2 animals compared to WT mice. Data represent fold up- or downregulation with p > 0.05. e. qPCR analysis of Klf15 mRNA in heart and liver of P2 and P7 Smn−/−;SMN2 mice compared to WT animals. Data represent mean ± SD, n = 3–4 animals per group, two-way ANOVA; **p < 0.01, ****p < 0.0001. f. Quantification of total S6 K1/total protein in triceps of P7 Smn−/−;SMN2 mice compared to healthy littermates. Total protein was visualized with Fast Green (FG) stain. Images are representative immunoblots. Data represent mean ± SD, n = 5–7 animals per group, two-tailed t-test; p = 0.0325. g. Quantification of phosphorylated (p)-S6 and total S6/total protein in triceps of P7 Smn−/−;SMN2 mice compared to healthy littermates. Total protein was visualized with Fast Green (FG) stain. Images are representative immunoblots. Data represent mean ± SD, n = 5–7 animals per group, two-tailed t-test; p-S6: p = 0.0024; total S6: p = 0.0024. h. Quantification of KLF15 protein/total protein in human gastrocnemius muscle samples from non-SMA control individuals and SMA Type I-III patients.