Using the VCP mutant mouse transgenic model of ALS and human post‐mortem tissue from sporadic ALS (sALS) patients, we previously showed that spinal motor neurons (MNs) exhibit reduced nuclear to cytoplasmic (N/C) ratios of FUS (Fused in Sarcoma) and SFPQ (Splicing factor Proline and Glutamine rich) proteins. The gene discussed is FUS; the disease is amyotrophic lateral sclerosis.