Genetic Rodent Models of Amyotrophic Lateral Sclerosis

Table 2

Overview of transgenic rodent models for motor neuron degeneration (Mendelian and typical FALS).

Disease	Gene product	Inheritance	Animal	Genetic modification	Reference
ALS1	Superoxide dismutase 1	Dominant	Mouse	genomic hSOD1 G37R	[29]
				genomic hSOD1 G85R	[30]
				genomic mSOD1 G86R	[31]
				genomic hSOD1 G93A	[32]
				genomic hSOD1 L126Z(stop)	[33]
				genomic hSOD1 L126delTT	[34]
				genomic hSOD1 Quad	[35]
				PrP; cDNA SOD1 G37R	[35]
				Thy-1; cDNA hSOD1 G93A	[36]
			Rat	genomic hSOD1 H46R	[37]
				genomic hSOD1 G93A	[37]
				genomic hSOD1 G93A	[38]
		Dominant/recessive	Mouse	genomic hSOD1 D90A	[39]
ALS6	FUS/TLS	Dominant (recessive)	Mouse	FUS/TLS KO	[40, 41]
ALS10	TDP-43	Dominant	Mouse	Prp; hTDP-43 A315T	[42]
				Thy-1; hTDP-43 WT	[43]
				PrP; hTDP-43 WT	[44]
				PrP; hTDP-43 A315T	[44]
				PrP; hTDP-43 M337V	[44]
				PrP; hTDP-43 WT	[45]
			Rat	TRE; hTDP-43 M337V and WT	[46]

hSOD1: human superoxide dismutase 1, mSOD1: mouse superoxide dismutase 1, FUS: fused in sarcoma, TLS: translocated in liposarcoma, KO: knockout, PrP: prion promoter, TRE: tTA-activated promoter.