• reduced life span

• early-onset Sodium Butyrate (SB, a HDAC inhibitor) treatment (from 4 weeks old) extends their average life span (13.7%)

• late-onset (from 12 weeks old) SB treatment extends the average life span (21.5%)

• neurons in the dentate cerebellar nucleus display somal atrophy

• extensive nuclear accumulation of Atrophin-1 and widespread nuclear inclusions in several brain regions (striatum, cortex, thalamus, red nucleus, dentate cerebellar nucleus, and Purkinje cells)

• progressively lose the capability to coordinate and balance in the rotarod test and the hanging wire test

• early-onset Sodium Butyrate (SB, a HDAC inhibitor) treatment (from 4 weeks old) delays the onset of motor impairments

• no neurodegeneration

• much weaker nuclear accumulation and nearly no inclusions

• normal life span (>60 week)