Table 1.

Overview of all reviewed murine models of SCA2

Transgenic line	Transgene	The age at onset of the motor dysfunction (weeks)	Reported pathological display	Reference
SCA2-58Q	Human ATXN2 gene with 58 CAG repeats	32	Significant loss of cerebellar PCs; progressive loss of calbindin-28k; disturbances of the electrophysiological functions	[21, 22, 124–127]
SCA2-75Q	Human ATXN2 gene with 75 CAG repeats	12	Specific cerebellar PC degeneration	[128]
SCA2-42KI	Murine ATXN2 gene with 42 CAG repeats	72	Reduced body weight; neuropathological mild and late-onset alterations	[129]
SCA2-127Q	Human ATXN2 gene with 127 CAG repeats	8	Ataxin-2 aggregates in the PCs; electrophysiological changes of the PCs; PC neuronal loss; abnormalities in the cellular gene expression	[130, 131]
BAC-SCA2-72Q	Human ATXN2 gene with 72 CAG repeats	16	Body weight loss; PC dendritic trees thinning; reduction of the calbindin and Pcp2 levels in the cerebellum	[132]