VCP and amyotrophic lateral sclerosis: These data were confirmed in 2010 by a study that found that knockdown of VCP or the overexpression of dominant-negative VCP (VCPR155H and VCPA232E mutations, involved in ALS/IBMPFD pathogenesis) lead to an accumulation of immature autophagic vesicles, often containing ubiquitin-positive contents and with acidified autophagosomes, defects found also when disease-associated VCP mutations were expressed (154).