Analysis of prion・gene ptomoter region using immortalized prionless cells

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Prions cause degenerative disease of the central nervous system in both humans and animals and are composed of the scrapie isoform of the prion protein (PrP^<SC>) (Prusiner, 1991 ; Chesebro et al., 1985 ; Oesch et al., 1985 ; Kretzschmar et al., 1986). The chromosomal PrP gene encodes both PrP^C and cellular isoform, PrP^C. The entire open reading frame (ORF) is contained within a single exon, which was considered to be separated from one or two exons encoding the 5' untranslated region of PrP mRNAs by a large intron. The cell surface localization of PrP^C together with its highly expression in the brains of neonatal hamsters suggested that PrP^C might function in cell recognition (Stahl et al., 1987 ; Mobley et al., 1998). Of particular interest are 5' upstream of a untranslated regions of so-called exon 1, which will work as a promoter for the transcription of PrP mRNA.These upstream region will help us to uncover the unknown promoter/enhancer system for PrP gene.