As previously found in the SOD1(G86R) ALS mouse model (Palamiuc et al., 2015), and consistently with higher PDH phosphorylation levels, ALS tibialis displayed significantly higher Pdk4 expression levels compared to control muscles (Figure 4C), whereas no significant change was found in the soleus (Figure 4D). Here, PDP1 is linked to amyotrophic lateral sclerosis.