A transgenic construct containing the human TATA box binding protein, TBP, containing a 71 polyQ repeat expansion, under the control of the mouse prion protein promoter was injected into fertilized FVB/N mouse eggs. A second founder line TBP-71Q-27 was established, in addition to line 71Q-16. The 71 polyQ-expansion is detected in the cerebral cortex and cerebellum by Western blot analysis.