Figure 1.

Representative behavioral analysis of Sandhoff mice treated from 6 weeks of age with 2,400 mg/kg per day of NB-DNJ. The data are from male siblings (n = 3 per group). The data are summarized as the means ± SD at each time point. (A) Performance of the mice in the bar-crossing test. (B) Performance of the mice in the inverted screen. (C) Activity levels on the inverted screen. ○, Untreated wild-type mice; ▵, untreated Sandhoff mice; and ▴, NB-DNJ-treated Sandhoff mice. Comparable data were obtained from female mice (data not shown).