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Figure 1.

HPLC-chromatograms of lyso-GM2 in the brain.

A wild-type mouse (W), an untreated Sandhoff mouse (S), and a Sandhoff mouse treated with the modified Hex B (S+E). Arrows indicate the peak of lyso-GM2.

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Figure 1 Expand

Figure 2.

MALDI-TOF-MS spectra of lyso-GM2.

Structure of lyso-GM2 (top) and its spectra obtained for the brain of a Sandhoff mouse (bottom).

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Figure 2 Expand

Figure 3.

Lyso-GM2 levels in the brains and plasma of mice.

Lyso-GM2 levels in brain tissues (A) and plasma (B). Wild-type mice (W,♦), untreated Sandhoff mice (S,▪), and Sandhoff mice treated with the modified Hex B (S+E,▴). Each line indicates the mean±SD. *P<0.05 (t-test).

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Figure 3 Expand

Figure 4.

GM2 levels in the brains of mice.

GM2 levels in brain tissues of wild-type mice (W,♦), untreated Sandhoff mice (S,▪), and Sandhoff mice treated with the modified Hex B (S+E,▴). Each line indicates the mean±SD. *P<0.05 (t-test).

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Figure 4 Expand

Table 1.

Plasma lyso-GM2 levels and hexosaminidase activities.

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Table 1 Expand