Remarkably, the transcriptional adaptation of Serpinb1a and Klk6 is substantial and strong already at pre-manifest stages of our SCA2 mouse model, suggesting early dysregulation of proteostasis control, long before the ATXN2 protein aggregates become microscopically detectable in spinal cord and cerebellar neurons25,76. The gene discussed is ATXN2; the disease is spinocerebellar ataxia type 2.