Table 1 Mouse models (SOD1, TARDBP, FUS and C9ORF72) used for ALS

SOD1-G93A [111, 112]

Human SOD1

ND

Yes

Yes

Yes

GOF

SOD1, vac

SOD1-A4V [113]

Human SOD1

No

ND

No

No

ND

None

SOD1-D90A [112]

Human SOD1

No

ND

Yes

Yes

GOF

SOD1, vac

SOD1-G85R [114, 115]

Human SOD

ND

ND

Yes

Yes

GOF

SOD1, UBI, LBHI

SOD1-G37R [116, 117]

Human SOD1

No

ND

Yes

Yes

GOF

SOD1, vac

hSOD1^WT [15]

Human SOD1

ND

ND

Yes

Yes

GOF

SOD1, vac

hSOD1^WT [118]

Human SOD1

ND

ND

Yes

No

GOF

SOD1, vac, UBI

TARDBP-A315T [119, 120]

BAC

ND

Yes

Yes

No

GOF

TDP43, UBI

TARDBP-A315T [119, 120]

mPrp

ND

ND

Yes

Yes

LOF

UBI

TARDBP-M337V [121]

mThy1

Yes

ND

Yes

Yes

GOF, LOF

TDP43, UBI, Variable

TARDBP-M337V [121, 122]

mPrp

Yes

Yes

ND

No

GOF, LOF

TDP43

TARDBP-Q331K [122, 123]

mPrp

Yes

ND

ND

No

GOF, LOF

Vac

TARDBP-Q331K [123, 124]

mPrp

Yes

ND

Yes

Yes

GOF

TDP43, p62, vac, UBI

TARDBP-G348C [119, 120]

BAC

ND

Yes

Yes

No

GOF

TDP43, UBI

hTDP43 [125, 126]

BAC, mPrp

ND

ND

Yes

Yes

GOF

TDP43, vac, UBI

FUS-R521C [127, 128]

Tau, mPrp

ND

ND

Yes

Yes

GOF, LOF

Diffused FUS

FUS-R521G [127, 128]

CAG

ND

ND

Yes

Yes

GOF, LOF

None

FUS-P525L [129]

Tau

ND

ND

Yes

Yes

GOF

Diffused FUS

hFUS [31, 130]

Tau, MPrp

No

ND

Yes

Yes

GOF

FUS, UBI

FUS^ΔNLS [131,132,133]

HR

Yes(C), ND(H)

ND

ND

No

GOF

FUS

C9ORF72 [100–1000] n [134]

BAC

No

No

No

No

ND

DPR, RNA foci

C9ORF72 [500] n [135]

BAC

No

No

No

No

ND

DPR), RNA foci

C9ORF72 [64] n [136, 137]

AAV-mediated somatogenesis

Yes(C), ND(H)

Yes

Yes

No

GOF

DPR, RNA foci, TDP43

C9ORF72 [147] n [136, 137]

AAV-mediated somatogenesis

Yes(C), ND(H)

Yes

Yes

No

GOF

DPR, RNA foci, TDP43

C9ORF72 [100] n [138]

self-complementary AAV9

Yes

Yes

No

No

GOF

RNA foci, TDP43

C9ORF72 [450] n [135]

BAC

Yes(C), ND(H)

Yes

No

No

GOF

DPR), RNA foci

C9ORF72 [500] n [139]

BAC

Yes

Yes

Yes

Yes

GOF

DPR, RNA foci, TDP43