Perry disease, a rare autosomal dominant neurodegenerative disorder, is characterized by parkinsonism, depression or apathy, unexpected weight loss, and central hypoventilation. Genetic analyses have revealed a strong association between point mutations in the dynactin I gene (DCTN1) coding p150 glued and Perry disease. Although previous reports have suggested a critical role of p150 glued aggregation in Perry disease pathology, whether and how p150 glued mutations affect protein aggregation is not fully understood. In this study, we comprehensively investigated the intracellular distribution of the p150 glued mutants in HEK293T cells. We further assessed the effect of co-overexpression of the wild-type p150 glued protein with mutants on the formation of mutant aggregates. Notably, overexpression of p150 glued mutants identified in healthy controls, which is also associated with amyotrophic lateral sclerosis, showed a thread-like cytoplasmic distribution, similar to the wild-type p150 glued . In contrast, p150 glued mutants in Perry disease and motor neuron disease caused aggregation. In addition, the co-overexpression of the wild-type protein with p150 glued mutants in Perry disease suppressed aggregate formation. In contrast, the p150 glued aggregation of motor neuron disease mutants was less affected by the wild-type p150 glued . Further investigation of the mechanism of aggregate formation, contents of the aggregates, and biological mechanisms of Perry disease could help develop novel therapeutics.