Table 2.

Animal models for spastin mutations

Organism	Mutation	Predicted spastin inactivation	Predicted level of mutated spastin	Onset/severity
Mouse	Homozygous deletion exons 5–7	100%	Not detected	Late onset, mild
Mouse	Homozygous splice site mutation	100%	Not detected	Late onset, mild
Bovine	Homozygous R560Q mutation	100%	Wild-type level	Early onset, very severe