Abstract
RE repeats encoded (RERE) was identified recently as a protein with high homology to the atrophin-1 protein, which appears to be causal in the hereditary neurodegenerative disorder termed dentatorubral-pallidoluysian atrophy (DRPLA) caused by an abnormal glutamine expansion. We have independently identified RERE in a search for genes localized to the translocation breakpoint 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, where it colocalizes with the promyelocytic leukemia protein in promyelocytic leukemia oncogenic domains (PODs). Overexpression of RERE recruits a fraction of the proapoptotic protein BAX to PODS: This observation correlates with RERE-induced apoptosis, which occurs in a caspase-dependent manner. These results identify RERE as a novel component of PODs and suggest an important role of RERE in the control of cell survival.
MeSH terms
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Alternative Splicing / genetics
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Apoptosis / genetics*
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Carrier Proteins / genetics
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Carrier Proteins / metabolism*
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Caspases / genetics
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Caspases / metabolism
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Cell Compartmentation / genetics
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Cell Nucleus / genetics
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Cell Nucleus / metabolism*
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Cell Survival / genetics
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DNA-Binding Proteins / genetics
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DNA-Binding Proteins / metabolism*
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Humans
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Kruppel-Like Transcription Factors
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Neoplasms / genetics
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Neoplasms / metabolism*
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Nerve Tissue Proteins / genetics
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Nerve Tissue Proteins / metabolism
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Neuroblastoma
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Neurodegenerative Diseases / genetics
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Neurodegenerative Diseases / metabolism*
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Promyelocytic Leukemia Zinc Finger Protein
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Protein Structure, Tertiary / genetics
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Proto-Oncogene Proteins / genetics
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Proto-Oncogene Proteins / metabolism
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Proto-Oncogene Proteins c-bcl-2*
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RNA, Messenger / metabolism
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Sequence Homology, Amino Acid
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Sequence Homology, Nucleic Acid
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Transcription Factors / genetics
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Transcription Factors / metabolism*
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Tumor Cells, Cultured
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bcl-2-Associated X Protein
Substances
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BAX protein, human
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Carrier Proteins
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DNA-Binding Proteins
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Kruppel-Like Transcription Factors
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Nerve Tissue Proteins
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Promyelocytic Leukemia Zinc Finger Protein
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Proto-Oncogene Proteins
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Proto-Oncogene Proteins c-bcl-2
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RERE protein, human
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RNA, Messenger
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Transcription Factors
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atrophin-1
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bcl-2-Associated X Protein
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ZBTB16 protein, human
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Caspases