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. Founder line TBP-71Q-16 was established. The 71 polyQ-expansion is detected in the cerebral cortex and cerebellum by Western blot analysis. (J:130775)