Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man.

By L. Fischer, D. Culver, P. Tennant, A. Davis, Minsheng Wang, A. Castellano-Sanchez, J. Khan, Meraida Polak, J. Glass. Experimental Neurology, 2004

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Synaptic energy use and supply. (2012, neutral): “In SOD1-FALS, NMJs are the first regions of the motor neurons to degenerate (Frey et al., 2000; Fischer et al., 2004).”
Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF (2006, background): “, more accessible synapses between motoneurons and muscles are also lost early on and in reproducible pattern”
The Effect of Training on Motoneuron Survival in Amyotrophic Lateral Sclerosis: Which Motoneuron Type is Saved? (2011, neutral): “This is exceptionally important in ALS because MNs retract from the neuromuscular junction before the MN cell body dies (Fischer et al., 2004).”
Axonal transport of TDP-43 mRNA granules in neurons is impaired by ALS-causing mutations (2014, neutral): “ALS has been described as a ‘‘distal axonopathy’’ because morphological abnormalities of the distal axon, including dismantling of the NMJ leading to denervation, are among the earliest pathological features (Fischer et al., 2004).”
The changing scene of amyotrophic lateral sclerosis (2013, background): “FIG4 is located in vacuolar membranes, regulates the phosphorylated state of PI(3,5)P2 and is essential for normal lysosome function41.”