To further study the neurodegeneration in specific affected brain regions in MJD, we generated an in vivo MJD genetic mouse model by ectopic expression of WT and mutant ATX-3 (80Q and C14A) with an enhanced green fluorescent protein (EGFP) tag in the substantia nigra pars compacta (SNpc) or striatum of p53+/+ and p53-/- mouse brain using lentiviral vectors (LV). Here, TP53 is linked to Spinocerebellar ataxia type 3.