Fig. 1.

Neurological deficits of HD sheep. Neurological response of sheep described by scoring from 1 to 5. Normal score is 1 and scores increase as responses worsen. A) Clinical rating scores of HD male and female were significantly higher than normal sheep. In particular, HD sheep showed significant deficit in strabismus and proprioception (*p < 0.05). (HD total n = 38, normal total n = 25, HD females n = 31, HD males n = 7, normal females n = 17, normal males n = 8). B) HD females showed sever signs of neurological deficit as compared to males (***p < 0.001). C) Cerebrospinal mHTT levels in females sampled at 9 and 10 years of age (HD n = 5, normal controls n = 4). mHTT levels for normal sheep was below the limit of detection (left, **p < 0.006). Three HD females showed an increase in mHTT level from 9 to 10 years of age (right).