Table 1.

Rodent models of ALS and FTLD–TDP based on TDP-43

ALS	Wegorzewska et al. (2009)	Wils et al. (2010)	Shan et al. (2010)	Xu et al. (2010)	Zhou et al. (2010)	Stallings et al. (2010)	Wang et al. (2010)	FTLD–TDP	Tsai et al. (2010)	Igaz et al. (2011)
Promoter	PrP	Thy-1	Thy-1.2	PrP	miniCMV/tetO	PrP	CBA	Promoter	CaMKII	CaMKII/tetO
TDP-43 form	A315T	WT	WT	WT	M337V	A315T	WT	TDP-43 form	WT	WT, ΔNLS
Onset of symptoms	3 months	0.5–14 months	0.5–3 months	3 weeks	3 weeks	1 month	2 weeks	Onset of symptoms	2–6 months	1–4 weeks
TDP-43 pathological modifications	No	Yes	No	Yes	Yes	Yes	No	TDP-43 pathological modifications	Yes	Yes
TDP-43-positive inclusions	no	yes	no	yes	Rare	Rare	no	TDP-43 positive inclusions	yes	Rare
TDP-43 fragments	Yes	Yes	no	Yes	Yes	Yes	ND	TDP-43 fragments	Yes	no
Motor neurodegeneration	Yes	Yes	no	Yes	ND	Yes	Yes	Forebrain neurodegeneration	Yes	Yes
Muscle atrophy	Yes	ND	Yes	No	Yes	Yes	Yes	Cognitive impairment	Yes	ND
Motor impairment	Yes	Yes	Yes	Yes	Yes	Yes	Yes	Motor impairment	Yes	Yes
Mortality	Yes	Yes	Yes	Yes	Yes	Yes	Yes	Mortality	Yes	No

Models of ALS have transgene expression in the spinal cord, which results in paralysis, motor neurodegeneration (either spinal motor neuron loss or corticospinal tract degeneration) or muscle atrophy. FTLD–TDP models have TDP-43 expression predominantly in the forebrain, resulting in cognitive dysfunction and neurodegeneration in the cortex or hippocampus. TDP-43 pathological modifications refer to either ubiquitination or phosphorylation, both of which were found in Zhou et al. (2010). PrP, prion protein; Thy, thymus cell antigen; CMV, cytomegalovirus; tetO, tetracycline operator; CBA, cytomegalovirus/chicken beta-actin; ND, not determined; NLS, nuclear localization signal; WT, wild-type.