Fig 1: Transcript and protein levels of the enzymes involved in ceramide production were studied in the cerebellum and spinal cord of Atxn2-CAG100-KIN and Atxn2-KO mice. (A) Expression levels of the de novo ceramide synthesis pathway components. (B) Expression levels of different sphingomyelinase isoforms catalyzing the breakdown of sphingomyelin species into ceramide. (C) Protein levels of the de novo ceramide synthesis (CERS1, CERS2) and sphingomyelin breakdown (aSMase, nSMase1, nSMase2) pathway components in >12 month old Atxn2-CAG100-KIN cerebellum tissue. Student’s t-test was used with Welch’s correction; T p < 0.1, * p < 0.05, ** p < 0.01, *** p < 0.001, **** p < 0.0001.