Decreased mitochondrial transport associated with cytoskeletal changes and/or impaired removal of altered mitochondria (mitophagy) and biogenesis of novel ones (mitobiogenesis) may similarly contribute to early pathological changes in axons and muscles of ALS and SMA (Wen et al. 2010; Natale et al. 2015; Ripolone et al. 2015; Xu et al. 2016). The gene discussed is SMN1; the disease is amyotrophic lateral sclerosis.