Table 3.

Overview of transgenic mouse models for motor neuron degeneration (atypical or rare FALS and candidate genes).

Disease	Gene product	Inheritance	Animal	Genetic modification	Reference
ALS2	Alsin	Recessive	Mouse	KO (exon 3)	[50]
				KO (stop codon in exon 3)	[51]
				KO (exon 3 and 4)	[52]
				KO (exon 4)	[53]
ALS8	VAPB	Dominant	Mouse	PrP; VAPB P56S	[54]
ALS	Dynactin	Dominant	Mouse	Knock-in G59S p150Glued	[55]
				Thy-1; G59 p150Glued	[56, 57]
CMT2E/1F	Neurofilament-L	Dominant	Mouse	NF-L L394P	[58]
NA	Peripherin	NA	Mouse	overexpression	[59]
NA	Vascular endothelial growth factor	NA	Mouse	VEGFδ/δ	[60]
FTDP-tau	tau	Dominant	Mouse	4R human tau	[61]
				R406W human tau	[62, 63]
				P301L human tau	[64]
				G272V, P301S human tau	[65]
				V337M human tau	[66]
				P301S human tau	[67]

PrP: prion promoter, CMT: Charcot-Marie-Tooth, FTDP: frontotemporal dementia with parkinsonism, and NA: not available.