Table 1 Prosaposin deficient mouse models
Genotype | Strain (%) | Life Span | Major CNS lipids (Accumulation level) | CNS phenotype | Prosaposin level in CNS (% WT) | References |
|---|---|---|---|---|---|---|
PS-/- | FVB | ~4 wks | 14 – 30 day GC (+ → +++) LacCer (+ → +++) | Ataxia, Tonic status epileptics | 0 | |
PS-NA | FVB | 32 wks | 4 – 22 wk GC (- → +) LacCer (- → +) | Ataxia, waddling gait | 45 | [7] |
4L/PS-NA | FVB (50) C57BL/6 (25) 129SvEvBrd (25) | 22 wks | 4 – 20 wk GC (+ → +++) LacCer (- → +) | Ataxia, waddling gait | 45 | [8] |