Fig. 1

CYP46A1 overexpression decreases the number of aggregates and the levels of soluble mutant HTT. a N2a cells were transfected with pEGFP-HttQ74, co-transfected with pEGFP-HttQ74 and pAAV-HA-CYP46A1, and co-transfected with pEGFP-HttQ74 and LacZ, highlighting the fusion protein with GFP, the nuclei labeled with DAPI and the expression of CYP46A1 by the labeling with HA tag. For each condition, 100 cells were randomly counted. b The number of cells with aggregates (white arrows) upon CYP46A1 overexpression was significantly reduced compared to control conditions (note the yellow arrows with expression of HTT-MUT without aggregates). c The size of HTT-MUT aggregates was smaller upon CYP46A1 overexpression relatively to both control conditions. d Western blot analysis probed with mouse anti-GFP depicting the soluble HTT-MUT (66 kDa) and tubulin as loading control. Densitometric analysis showed that CYP46A1 expression leads to a significant reduction of the soluble HTT-MUT (e). Values are expressed as mean ± SEM. (a–c, n = 5 independent experiments; d–e, n = 4 independent experiments; One-way ANOVA with Bonferroni’s multiple comparisons test, *P < 0.05; **P < 0.001; ****P < 0.00001)