Table 2.

Overview of mouse models for SCA 1.

	B05 transgenic mice (B05)	Sca1 154Q/2Q mice (154Q/2Q)
Phenotype	Motor incoordination, ataxia (12 weeks), no cognitive impairment	motor incoordination, muscle wasting, cognitive impairment, memory deficits
Physical Onset	5 weeks	7–8 weeks
Course of the disease	Normal life span	Premature, 35–45 weeks
Neuropathology	PC loss, Bergmann glial proliferation, shrinkage and gliosis of molecular layer	Reduced dendritic arborization of PCs (early stage)
		Neuronal intranuclear inclusions and PC loss (advanced disease)
	Purkinje neuron dendritic and somatic atrophy	Hypocampal synaptic dysfunction but no significant loss
Mechanism	Overexpression of polyQ-ATXN1 (82 CAG repeats) under the control of Purkinje Cell pcp2 promoter	Expanded repeat of 154 CAGs was inserted into the mouse Sca 1 locus (knock-in)
References	Burright et al., 1995; Clark et al., 1997	Watase et al., 2002