Table 6 FUS animal models

Mice

KO

n/a

nd

nd

nd

nd

24 h death, chromosomal abnormality, sterility

nd

[198]

KO

n/a

nd

nd

nd

Y

No motor phenotype, hyperactivity behavior

nd

[199]

hFUS^WT

mPrp

4 weeks

10–13 weeks

Y

N

Tremors, weight loss, deficit in rotarod, increase cytoplasmic FUS signal, spinal MN loss, NMJ loss

Y

[200]

hFUS^WT, R521G

CAG

10

30

N^a

Y

More lethality in FUS^WT, no MN loss in lateral column, MA, NMJ loss, reduced social interaction and motor performance

Y

[201]

Rats

hFUS^WT, R521C^b

TRE

27–48

33–55

Y

Y

Few MN loss, MA, NMJ loss, ubiquitin inclusions, no FUS inclusions

Y

[202]

Fruit flies

hFUS^WT, R524S, P525L

OK371-Gal4

nd

nd

nd

nd

Large MN, decreased locomotor function, NMJ loss

nd

[203]

hFUS^WT, R518K, R521C, R521H

OK371-Gal4/elav-GS

10

~17

nd

nd

Decreased locomotor function, no NMJ loss, more cytosolic FUS in mutant,

nd

[204]

Nematodes

hFUS^WT, R514G, R521G, R522G, P525L, FUS513, FUS501

Prgef-1

3

8.1–9.7

Y

nd

NCI of mutant FUS, worst phenotype in R522G, P525L, FUS513 and FUS501

nd

[206]

S57Δ

unc-47

12–13

normal

Y

nd

MN loss, FUS insoluble aggregates

nd

[207]

Zebrafish

R521C, R521H, S57Δ

mRNA

48 hpf

nd

nd

nd

Reduced swimming at TEER, NMJ loss

nd

[208, 209]