Table 1 Therapeutic outcomes for CuATSM across multiple mouse models of amyotrophic lateral sclerosis.
Mouse model | Genetic background | Commencement of treatment | Daily dose (mg/kg) | Administration route | Increase in survival | Body weight | Motor function | Neurological score | Study |
|---|---|---|---|---|---|---|---|---|---|
Low copy SOD1-G93A | Congenic; C57BL/6 | 140 days (presymptomatic) | 30 (5 days per week) | Oral | 14% | Delayed onset | Delayed onset/slowed progression | Delayed onset | 23 |
Low copy SOD1-G93A | Congenic; C57BL/6 | 200 days (symptomatic) | 30 (5 days per week) | Oral | 10% | NR | Slowed progression | NR | 23 |
High copy SOD1-G37R | Congenic; C57BL/6 | 40 days (presymptomatic) | 10 (7 days per week) | Oral | 8% | NR | Delayed onset/slowed progression | NR | 24 |
High copy SOD1-G37R | Congenic; C57BL/6 | 40 days (presymptomatic) | 30 (7 days per week) | Oral | 18% | NR | Delayed onset/slowed progression | NR | 24 |
High copy SOD1-G37R | Congenic; C57BL/6 | 40 days (presymptomatic) | 60 (7 days per week) | Oral | 26% | NR | Delayed onset/slowed progression | NR | 24 |
High copy SOD1-G37R | Congenic; C57BL/6 | 149 days (symptomatic) | 60 (7 days per week) | Oral | 12% | NR | Slowed progression | NR | 24 |
High copy SOD1-G37R | Congenic; C57BL/6 | 40 days (presymptomatic) | 30 (7 days per week) | Oral | 18% | NR | Delayed onset/slowed progression | NR | 17 |
High copy SOD1-G93A | Mixed; B6SJL | 5 days | 200 (7 days per week) | Transdermal | 25% | Slowed progression | NR | Delayed onset | 18 |
High copy SOD1-G93A | Mixed; B6SJL | 50 days | 200 (7 days per week) | Transdermal | 19% | Slowed progression | NR | Delayed onset | 18 |
High copy SOD1-G93A × CCS | Mixed; B6SJL | Prenatal | 60 (7 days per week) | Transdermal | 2800% | Slowed progression | NR | NR | 18 |
High copy SOD1-G93A | Mixed; B6SJL | 50 days (presymptomatic) | 100 (7 days per week) | Oral | 9% | NR | Delayed onset/slowed progression | Delayed onset | 20 |
High copy SOD1-G93A | Mixed; B6SJL | 50 days (presymptomatic) | 30 (7 days per week) | Oral | *Improved survival by 5.5 days | Slowed progression | NR | Delayed onset | 45 |
Neurotoxin; β-sitosterol β-d-glucoside | Outbred;CD-1 | 63 days | 30 (5 days per week) | Transdermal | NR | No significant difference | Slowed progression | Improved leg extension reflex | 47 |
