Table 1 Summary of therapeutic outcomes for CuII(atsm) across multiple mutant SOD1 mouse models of ALS.

From: CuII(atsm) improves the neurological phenotype and survival of SOD1G93A mice and selectively increases enzymatically active SOD1 in the spinal cord

Study

Mouse model

Genetic background

Age when treatment commenced

Daily dose (mg kg−1 body weight)

Administration route

Increase in survival #

Soon et al.9

Low copy SOD1G93A

Congenic; C57BL6

140 days

30 (5 days week−1)

Oral

14%

Soon et al.9

Low copy SOD1G93A

Congenic; C57BL6

200 days

30 (5 days week−1)

Oral

10%

McAllum et al.7

High copy SOD1G37R

Congenic; C57BL6

40 days

10 (7 days week−1)

Oral

8%

McAllum et al.7

High copy SOD1G37R

Congenic; C57BL6

40 days

30 (7 days week−1)

Oral

18%

McAllum et al.7

High copy SOD1G37R

Congenic; C57BL6

40 days

60 (7 days week−1)

Oral

26%

McAllum et al.7

High copy SOD1G37R

Congenic; C57BL6

149 days *

60 (7 days week−1)

Oral

12%

Roberts et al.8

High copy SOD1G37R

Congenic; C57BL6

40 days

30 (7 days week−1)

Oral

18% §

Williams et al.10

High copy SOD1G93A

Mixed; B6SJL

5 days

200 (7 days week−1)

Transdermal

25%

Williams et al.10

High copy SOD1G93A

Mixed; B6SJL

50 days

200 (7 days week−1)

Transdermal

19%

Williams et al.10

High copy SOD1G93A x CCS

Mixed; B6SJL

Prenatal

60 (7 days week−1)

Transdermal

2,800%

Present study

High copy SOD1G93A

Mixed; B6SJL

50 days

100 (7 days week−1)

Oral

9%

  1. #Increase in mean survival relative to sham treated mice.
  2. *Treatment commenced after individual mice lost 20% of their basal pre-symptom functionality on the rotarod test for locomotive function and 149 days is the mean age at which all mice included in the study reached this phenotypic criterion.
  3. §Performed concurrently with the McAllum et al. study. These survival data were presented in the McAllum et al. paper and therefore not re-published in the Roberts et al. paper which instead reported other therapeutic outcomes and mechanism of action data.