Figure 1

Huntingtin expression in YAC18/128 mice. YAC18 and YAC128 mice were crossed to generate YAC18/128 mice. A. To confirm high levels of htt over-expression in YAC18, line 212 mice we performed Western blots on whole brain lysates. We found that line 212 mice have 2.4 times the levels of wild-type htt as WT mice (WT: 0.386 ± 0.014 arbitrary units, YAC18: 0.942 ± 0.056 arbitrary units, p = 0.02). B. Total htt and human htt levels were assessed by Western blotting with MAB2166 and HD650 antibody respectively. The human specific HD650 antibody was used to detect htt expressed from the YAC transgenes in YAC18, YAC128 and YAC18/128 mice. Western blots performed with HD650 antibody indicate that YAC18/128 mice express both wild-type htt and mutant htt from YAC transgenes. As expected WT mice express no human htt, YAC18 mice express only wild-type human htt and YAC128 mice express only mutant human htt. Examination of total htt levels with MAB2166 antibody reveals that all mice express similar levels endogenous wild-type htt. C. Quantification of protein expression reveals that YAC18/128 mice express mutant htt at the same level as YAC128 mice (YAC128: 3389 ± 197 arbitrary units, YAC18/128: 3303 ± 316 arbitrary units, p = 0.8). N = 3 mice per group. Error bars indicate standard error of the mean. A.U. = arbitrary units.