Human RERE is localized to nuclear promyelocytic leukemia oncogenic domains and enhances apoptosis

RE repeats encoded (RERE) was identified recently as a protein with high ho mology to the atrophin-1 protein, which appears to be causal in the heredit ary neurodegenerative disorder termed dentatorubral-pallidoluysian atrophy (DRPLA) caused by an abnormal glutamine expansion. We have independently id entified RERE in a search for genes localized to the translocation breakpoi nt region at chromosome 1p36.2 in the neuroblastoma cell line NGP. Here we show that neuroblastoma tumor cell lines display reduced abundance of RERE transcripts. Furthermore, we detected RERE protein mainly in the nucleus, w here it colocalizes with the promyelocytic leukemia protein in promyelocyti c leukemia oncogenic domains (PODs), Overexpression of RERE recruits a frac tion of the proapoptotic protein BAX to PODs. This observation correlates w ith RERE-induced apoptosis, which occurs in a caspase-dependent manner. The se results identify RERE as a novel component of PODs and suggest an import ant role of RERE in the control of cell survival.