Tg(Pcp2-Prnp)F332Zbz

Mice bearing this transgene express a truncated mouse prion protein (PRNP) lacking amino acids 32 through 134 under the 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 its amino terminus; these presumably are removed along with the PRNP N-terminal leader sequence. Quantitative PCR analysis suggests this line carries ~130 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)