Additionally, in an HD transgenic mouse model (R6/2) expressing exon 1 of the human HTT gene carrying 141–157 CAG repeats [124], an overexpression of ceramide species C20:0, C22:0, C24:0, and C24:1 was seen in the striatum, while ceramide C24:0 was found to be upregulated in the cortex of these mice [118]. The gene discussed is HTT; the disease is Huntington disease.