SMN2 and amyotrophic lateral sclerosis: In this frame, it would be interesting to investigate the disease-modifying effects of compounds, which are known to increase SMN levels while preventing both motor neuron loss, and motor impairment associated with mitochondriopathy and axon clogging in ALS and SMA models (Feng et al. 2008; Fornai et al. 2008a, b; Harahap et al. 2012; Natale et al. 2015; Biagioni et al. 2017).