UBQLN2 and amyotrophic lateral sclerosis: Transgenic rats expressing UBQLN2P497H recapitulated features of UBQLN2-related ALS, including protein aggregates that contained the mutant UBQLN2, motor neuron loss, cortex structure deformations, and progressive accumulation of p62 and LC3-II proteins within the aggregates (Wu et al., 2015; Huang et al., 2016).