Tg(Prnp-UBQLN2*P506T)#Hlp

The transgene contains the following elements: the mouse prion protein gene promoter and exon 1, intron 1 and exon 2, sequence coding for a FLAG tag in-frame with the human Ubqln2 cDNA that has been modified to change proline codon 506 into a threonine codon (p.P506T), and Prnp exon 3 and downstream sequence. The mutation is associated with familial frontotemporal dementia/amyotrophic lateral sclerosis (FTD/ALS). (J:267076)