Figure 2.

(A) Graphical representation of HPTLC analysis of brain and liver G_M2 and G_A2 in Sandhoff mice in the presence or absence of NB-DNJ. Mice were treated from weaning at 3 weeks with 4,800 mg/kg per day of NB-DNJ, and GSL profiles were compared at 16 weeks of age relative to untreated age-matched controls. The HPTLC plates were analyzed by densitometry in relation to authentic GSL standards. (B) Graphical representation of HPTLC analysis of brain region G_M2 and G_A2 content at the end stage of untreated controls (125 days) and NB-DNJ-treated Sandhoff mice (170 days). CC, cerebral cortex; THP, thalamus/hypothalamus; MPM, midbrain, pons, and medulla; CBL, cerebellum; SPN, spinal cord. (C) Comparison of G_M2 and G_A2 levels in whole brain and liver at the end stage of diseases in untreated (125 days) and NB-DNJ-treated mice (170 days). The data are presented as the mean ± SD for three mice per group. Solid bars depict data from untreated mice, and open bars depict data from NB-DNJ-treated mice. Representative data are shown. The data set shown is from male mice, and comparable data were obtained from females (data not shown).