PQBP-1 is a polyglutamine tract binding protein implicated in transcription. We previously reported that PQBP-1 and mutant ataxin-1, product of the spinocerebellar atrophy type 1 (SCA1) causative gene, cooperatively induce cell death in culture cells. Simultaneously, we showed that mutant ataxin-1 promoted interaction between PQBP-1 and RNA polymerase II and enhanced repression of the basal transcription by PQBP-1. In this study, we have examined the effects of overexpression of PQBP-1 to the primary-cultured cerebellar neurons. Our results indicate that overexpression of PQBP-1 inhibits the basal transcription in cerebellar neurons and increases their vulnerability to low potassium conditions. (C) 2002 Elsevier Science (USA). All rights reserved.