Analysis of prion・gene ptomoter region using immortalized prionless cells

朊病毒在人和动物中引起中枢神经系统的退行性疾病，并且由朊病毒蛋白（PrP β 2）的瘙痒病同种型组成<SC>（Prusiner，1991 ; Chesebro等人，1985 ; Oesch等人，1985 ; Kretzschmar等人，1986年）。染色体PrP基因编码PrP^C和细胞同种型PrP^C。整个开放阅读框（ORF）包含在单个外显子内，该外显子被认为通过大内含子与编码PrP mRNAs的5'非翻译区的一个或两个外显子分开。PrP^C的细胞表面定位及其在新生仓鼠脑中的高表达表明PrP^C可能在细胞识别中起作用（斯塔尔等人，1987 ; Mobley等人，1998年）。特别值得关注的是PrP基因外显子1的5'上游非翻译区，它将作为PrP mRNA转录的启动子，这些上游区域将帮助我们发现未知的PrP基因启动子/增强子系统。

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.