Tg(Pcp2-Prnp)F13Zbz

Mice bearing this transgene express a truncated mouse prion protein (PRNP) lacking amino acids 32 through 134 under transcriptional control of the regulatory elements of the Purkinje cell protein 2 (L7) gene. A cDNA encoding the modified PRNP and extending to the EcoR1 site in the 3' UTR was inserted into a cloned mouse genomic DNA segment containing the entire Pcp2 coding region, 4 kb of 5' flanking sequence and 2 kb of 3' flanking sequence. Insertion occurred at the BamH1 site in the fourth (third coding) exon, so the initial translation product has 80 amino acids of PCP2/L7 joined to the amino terminus of PRNP; these presumably are removed along with the PRNP N-terminal leader sequence. Quantitative PCR analysis suggests this line carries ~60 transgene copies. Immunohistochemical analysis demonstrated high levels of expression of the truncated PRNP in cell bodies, axons and dendrites only of Purkinje cells of transgenic mice lacking endogenous PRNP expression; no expression is seen in granule cells. (J:47034, J:83952, J:173517)