Table III.
Biochemical and cell biological consequences of GSL storage in the thymus of the Sandhoff disease mouse
| Control | Sandhoff | Fold elevation | ||
|---|---|---|---|---|
| HPLC analysis of GSLs | aGM1a | 28.3 ± 2.9 | 24.8 ± 2.1 | 0.89 |
| aGM1b | 150.3 ± 24 | 133.7 ± 15.8 | 0.89 | |
| aGA1 | 121.7 ± 12.6 | 118.6 ± 12.8 | 0.97 | |
| aGM2 | 8.3 ± 1.5 | b17.9 ± 3.8 | 2.15 | |
| aGA2 | 34 ± 4.89 | b166.9 ± 24.8 | 4.91 | |
| aGM3 | 5.8 ± 1.05 | b2.14 ± 0.75 | 0.36 | |
| Flow cytometry | cLysoTracker | 100 ± 5.3 | b211.3 ± 21.3 | 2.11 |
a
Levels of GSL species in total thymic extract from 10–12-day-old Sandhoff and control mice (pg/mg protein), as determined by HPLC (reference 41).
b
Statistical significance using the t test (P ≤ 0.05).
c
A relative measure of the total acidic late endosomal/lysosomal compartment was made using LysoTracker staining. Flow cytometry was used to analyze LysoTracker-stained thymocytes (relative fluorescence intensity), gating on the lymphoid population using forward and side scatter parameters.