In transgenic mice, expression of the Q92 ataxin-7 in all CNS neurons, except for Purkinje cells, led to severe Purkinje cell degeneration, development of gait ataxia, and formation of truncated ataxin-7 nuclear aggregates that correlated with disease phenotype onset and even to premature death [248]. The gene discussed is ATXN7; the disease is Gait ataxia.