Table 3.

The major advantages and downsides of SCA2 mouse models.

Mouse models	Representative line	Advantages	Disadvantages
Q58	Q58-11	Robust, well-characterized behavioral impairments and neuropathology;	Unable to study other brain regions: targeted expression to PCs;
		Suitable to study PCs dysfunction;	Non-physiological expression levels of ataxin-2—overexpression;
		Practical for research into new therapeutic strategies;	Pronuclear injection: random integration;
Q75	F066	Ubiquitous expression of mutant ataxin-2;	Pronuclear injection: random integration;
		Integrated only 1 or 2 copies of the transgene;	Lack of control transgenic line;
		Early onset of symptoms and robust motor impairment—practical in testing for new therapies;	No other symptom besides motor incoordination;
Q42 KI	CAG42	Very faithful to the human condition in terms of physiological expression levels and age of onset;	Mild ataxia phenotype and neuropathology with late onset of symptoms;
		Ideal to study early differential gene expression levels;	Unpractical for testing new therapies that alleviate symptoms;
		Potential to find disease biomarkers;
		Targeted insertion;
Q127	ATXN2Q127	Very robust impairments in behavior and neuropathology with early onset of symptoms;	Unable to study other brain regions: targeted expression to PCs;
		Suitable to study electrophysiological dysfunctions in PCs and testing new therapies;	Decreased faithfulness to the human disease;
			Pronuclear injection: random integration;
BAC-Q72	BAC-ATXN2-Q72	The entire gene (introns included) was inserted into the mouse genome: suitable to study RNA-related mechanisms;	No other symptoms besides motor incoordination;
		Ubiquitous expression of mutant ataxin-2;	Mild neuropathology;

The major advantages and disadvantages of SCA2 mouse models. For each mutant mouse line, the most notorious upsides and downsides are given to facilitate the choice of the most adequate model addressing different biological questions.