In parallel with the study in human subjects, mouse Bcl2, Bax and Trp53 transcript and protein levels were assessed in blood of pre-symptomatic 9 MO Q84 transgenic mice, and in the pons and cerebral cortex of both pre-symptomatic 9 MO and symptomatic 18 MO Q84 mice [10,30] to evaluate whether this widely used MJD mouse model replicates the findings observed in MJD subjects. Here, BCL2 is linked to Machado-Joseph disease.