Table 2 SOD1 animal models

Mice

hSOD1^WT

58–70

normal

N

nd

Mitochondria vacuolization and swelling,, spinocerebellar axonal degeneration, 20-30 % MN loss

Y

[69]

hSOD1^WT

36

52

Y

nd

SOD1 inclusions, vacuolization, MN loss, glial cell aggregates

Y

[71]

A4V

>85

normal

N

N

nd

N

[70]

A4V/SOD1^WT

35

48

Y

nd

SOD1 inclusions, MN degeneration

Y

[70]

G37R

15–17

25–29

Y

Learning deficit

MBV, LMN first affected, raised somatosensory thresholds

Y

[76, 243]

H46R

20

24

Y

nd

LBHI, ubiquitin, SOD1 inclusions

Y

[244]

H46R/H48Q

17–26

nd

Y

nd

HI, ubiquitin

nd

[245]

H46R/H48Q/

35–52

nd

Y

nd

Fibrillary SOD1-ubiquitin inclusions

Y

[246]

H63G/H120G

D83G^a

15

70–84

N

nd

Sensory deficit, tremors, 20 % LMN and UMN loss

Y

[78]

L84V

21–26

26–30

Y

nd

nd

nd

[247]

G85R

35–43

37–45

Y

nd

Rapid progression, SOD1-ubiquitin inclusions in neurons and astrocytes

Y

[248]

G85R/SOD1^WT

16–21

23–30

Y

nd

SOD1 aggregates

Y

[249]

G86R^b

13–17

17

Y

nd

Rapid progression (5 days)

nd

[250]

D90A

52

61

Y

nd

Distended bladder, SOD1 inclusions, MN loss

Y

[75]

G93A

13–17

17–26

Y

Y

MN loss, SOD1 aggregates, NMJ loss before onset

Y

[61, 77]

G93A/SOD1^WT

20–23

25–30

Y

nd

Vacuoles, MN loss

Y

[69]

Thy1.2-G93A^c

54- >104

62- >104

N

nd

SOD1 aggregates

Y

[79]

L126Z

28–44

47

Y

nd

Eosinophilic inclusion, MN loss, ubiquitin inclusions

Y

[70, 251]

G127X

35

36

Y

nd

Rapid disease course, SOD1-ubiquitin inclusions

Y

[252]

Rats

H46R

20

24

Y

nd

MN loss, LBHI, SOD1-ubiquitin aggregates

Y

[253]

G93A

16

17

Y

nd

MN loss, vacuoles, SOD1-ubiquitin inclusions

Y

[253, 254]

Dogs

T18S

7 years

21 months^d

Y

nd

SOD1 aggregates No neuronal cell body loss, UMN and LMN signs, sensory impairment

Y

[86]

E40K

>5 years

6 months–3 years^d

Y

nd

Y

[85]

Zebrafish

A4V^e

30 h

nd

N

nd

Motor axonopathy and abnormal branching

nd

[90]

G37R^e

30 h

nd

N

nd

nd

[90]

G93A^e

30 h

nd

N

nd

nd

[90]

G93A

20–60

nd

N

nd

Increase time resting but no swim speed change, NMJ loss, 50 % MN loss

nd

[91]

G93R^f

12 months

18–27 months

partial

nd

NMJ defects, MN loss, swimming incapacity, vacuolated mitochondria

nd

[92]

Fruit flies

hSOD1^WT

3

normal

N

nd

Loss of climbing, no MN loss, decrease synaptic transmission in giant fiber motor pathway

HSP70 stress response

[93]

A4V

4

normal

N

nd

[93]

G85R

2

normal

N

nd

[93]

D83S

4

normal

N

nd

Mitochondrial pathology, decreased physical activity

nd

[255]

Nematodes

hSOD1^WT

10 days

10–20 D

Y

nd

Reduction in thrash number

nd

[95]

G85R

10 days

10–20 D

Y

nd

Forward movement defect, SOD1 inclusions

nd

[95]

G93A

2 days

nd

Y

nd

SOD1 inclusions in MN, axons guidance defects

nd

[256, 257]

C6S/C57S/

normal

normal

N

nd

No phenotype

nd

[95]

C111S/C146S

Pigs

G93A

12

normal

N

nd

MN loss at 8 months, Intra-nuclear SOD1-ubiquitin inclusions, running deficit, fibrillation potentials and positive sharp waves at EMG

Y

[99]