Figure 1.

CA150 overexpression protects primary striatal cells from mutant htt toxicity. A, Eight weeks after infection, neuronal dysfunction was measured by Neu-N immunohistochemistry. Neu-N expression was restored after htt171-82Q and CA150 infection compared with cells infected with htt171-82Q alone (∗∗p < 0.005; average rescue is 83.3%). Data are mean ± SD (n = 6) expressed as percentages of control, namely htt171-19Q (27.2 ± 9 Neu-N-positive cells/field of view). The mean rescue of htt171-82Q-infected cells by CA150 was 83.3%. B, CA150, and CA150 deleted for the (Gln-Ala)₃₈ repeat (CA150ΔQA), did not alter PGK-driven expression as assessed by GFP immunoblot analysis 6 weeks after infection. C, No noticeable difference of htt transgene expression by CA150 or CA150ΔQA overexpression was observed 3 weeks after infection, as tested by immunoblot for the myc tag of htt.