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	[1,10]
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]