In “wobbler” mutant mice, which develop a progressive ALS-like motor neuron degeneration phenotype due to homozygous Vps54 Q967L mutations (impairs endosome-Golgi trafficking), giant endosomal vesicles were observed in motor neurons, which were Rab7-positive and mostly LC3-negative, mimicking our observations. The gene discussed is VPS54; the disease is amyotrophic lateral sclerosis.