Table 2.

Overview of transgenic TDP-43 overexpression mice

	Transgene	Promoter	Expression (upon mTDP-43)	Ubiquitin inclusions	TDP-43 inclusions	C-terminal fragments	Ref.
Wegorzewska et al. (2009)	p.A315T	PrP	3-fold	Present (brain and spinal cord)	Absent	25-kDa and 35-kDa CTFs (progression with disease)	[22]
Wils et al. (2010)	Wild-type	Thy-1.2	2-fold	Present (brain and spinal cord)	Rare (brain and spinal cord)	25-kDa and 35-kDa CTFs (progression with disease)	[24]
Tsai et al. (2010)	Wild-type (mTDP-43)	CaMKII	2-fold	15–20 % of neurons (brain)	15–20 % of neurons (brain)	25-kDa and 35-kDa CTFs	[23]
Shan et al. (2010)	Wild-type	Thy-1	3.6-fold (males)	Rare (brain and spinal cord)	Present	n.d.	[19]
			1.3-fold (females)
Xu et al. (2010)	Wild-type	PrP	2.5-fold	Absent (increased reactivity)	Rare (brain and spinal cord)	25-kDa and 35-kDa CTFs	[25]
Xu et al. (2011)	p.M337V	PrP	2.7-fold	Absent (increased reactivity)	Rare (brain and spinal cord)	25-kDa and 35-kDa CTFs	[28]
Stallings et al. (2010)	Wild-type	PrP	Up to 8-fold	Present (brain and spinal cord)	Rare (brain and spinal cord)	25-kDa and 35-kDa CTFs	[20]
	p.M337V
	p.A315T
Igaz et al. (2011)	Wild-type	CaMKIIα	0.4–1.7-fold	Rare (brain)	Rare (<1 %)	Absent	[18]
Swarup et al. (2011)	Wild-type	TDP-43	3-fold	Present (mutants)	Present (mutants)	25-kDa and 35-kDa CTFs (mutants)	[27]
	p.A315T
	p.G348C
Tian et al. (2011)	Wild-type	CAG	n.d. (22 cDNA copies)	Absent	Absent	n.d.	[21]
	p.M337V
Cannon et al. (2012)	Wild-type	CaMKIIα	Up to 3-fold	Present	Present	25-kDa and 35-kDa CTFs	[26]
Janssens et al. (present paper)	p.M337V	Thy-1.2	Up to 1.8-fold	Present (only in brain)	Phospho TDP-43 granules (brain)	25-kDa and 35-kDa CTFs (progression with disease)	/
	(Cfr. wild-type; Wils et al., 2010)

n.d. not determined