However, in CSTB knock-out mice, ataxia, myoclonic seizures, rapid cerebellar degeneration and apoptosis are noted [47] and are associated with significant increases in transcription of cathepsin S and markers of glial activation [48], themselves consistent with reactive changes suggestive of widespread cortical and subcortical grey matter damage [49]. The gene discussed is CSTB; the disease is cerebellar ataxia.