KLK6 and spinocerebellar ataxia type 2: 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.