UBQLN2 and amyotrophic lateral sclerosis: UBQLN2(P497H) transgenic mice, causes amyotrophic lateral sclerosis (ALS) and frontotemporal type of dementia, had the feature of a dendritic spinopathy with protein aggregation in the dendritic spines and an associated decrease in dendritic spine density and synaptic dysfunction, related to impaired protein degradation (Gorrie et al., 2014).