RANBP2 and amyotrophic lateral sclerosis: For example, RANBP2 protein levels are lower in hippocampal extracts from ME7 mice, a prion model for human neurodegenerative disease [38]; additionally, RANBP2 down-regulation in motor neurons disrupts multiple essential cellular pathways and directly induces ALS-like neurodegenerative pathology in vivo [39].