Table 1 Gba1 mutants and Saposin C deficiency models.
Mouse models | Life span | Neuronal phenotype | Storage cells | GCase activity | Glucosylceramide Increase |
|---|---|---|---|---|---|
Combined mutants | vs. Gba1 mutant | (Brain, Viscera) | |||
9V;C* | <1 day | un | un | decreased | Yes, Yes |
0S;C* | <1 day | un | un | decreased | un, Yes |
4L;C* | 7 weeks | Yes | un | decreased | Yes, Yes |
9H;C* | ~13 months | Yes | Yes | same low level | Yes, Yes |
Gba1 and Saposin C mutants # | %WT | (Brain, Viscera) | |||
9V/9V (9V) | ~24 months | ~2 years | un | ~5 | un, Yes |
0S/0S (0S) | <1 day | un | un | ~10 | un, Yes |
4L/4L (4L) | ~24 months | un | un | ~10 | Yes, Yes |
9H/9H (9H) | ~24 months | un | un | ~5 | un, Yes |
WT;C* | ~24 months | Yes | un | ~40 | un, un |
