In agreement with ALS-associated mitochondrial damage leading to PINK1/Parkin-mediated halting of mitochondrial transport, decreased levels of Miro1 have been reported in SOD1G93A and TDP-43M337V transgenic mice as well as in the spinal cord of ALS patients (Zhang et al., 2015) while down-regulation of either PINK1 or Parkin partially rescued the locomotive defects and enhanced the survival rate in transgenic flies expressing FUS (Chen et al., 2016). Here, RHOT1 is linked to amyotrophic lateral sclerosis.