Table 4.
Therapeutic approaches using CoQ10 in mouse models of polyQ diseases
| Drug | Route/Dose | Model | Therapeutic outcomes | Reference | |
|---|---|---|---|---|---|
| CoQ10/remacemide | Food supplemented with 0.2 % of CoQ10 (500 mg/kg/day) and 0.007 % of remacemide (17.5 mg/kg/day) | N171-82Q (HD) | ✓ | Improved rotarod performance; decreased body weight loss rate | Schilling et al. 2001 [153] |
| × | No change in survival; no change in inclusion formation | ||||
| CoQ10 | Powdered food supplemented with 0.2 % of CoQ10 (500 mg/kg/day) | N171-82Q (HD) | ✓ | Improved rotarod performance | Schilling et al. 2004 [28] |
| × | Shortened life span (powdered food formulations effect) | ||||
| CoQ10/remacemide | Food supplemented with 0.2 % of CoQ10 (400 mg/kg/day) and 0.007 % of remacemide (14 mg/kg/day) | N171-82Q (HD) | ✓ | Attenuated body weight loss; prolonged life span | Ferrante et al. 2002 [154] |
| CoQ10/remacemide | Food supplemented with 0.2 % of CoQ10 (400 mg/kg/day) and 0.007 % of remacemide (14 mg/kg/day)—separate or combined | R6/2 (HD) | ✓ | Improved rotarod performance; attenuated body weight loss; prolonged life span; delayed brain weight loss; attenuated gross brain atrophy and ventricular enlargement; attenuated neuronal atrophy; reduced number of striatal aggregates | Ferrante et al. 2002 [154] |
| CoQ10 | Food supplemented 1,000, 5,000, 10,000, or 20,000 mg/kg/day (Chemco) | R6/2 (HD) | ✓ | Prolonged life span (dose dependent); reduced body weight loss; improved rotarod phenotype; increased forelimb strength; attenuated gross brain size decline and striatal atrophy; reduced aggregate formation | Smith et al. 2006 [187] |
| CoQ10 | Food supplemented 400, 1,000, and 2,000 mg/kg/day (Tishcon) | R6/2 (HD) | ✓ | Prolonged life span (dose dependent); reduced body weight loss; improved rotarod phenotype | Smith et al. 2006 [187] |
| CoQ10/minocycline | Food supplemented with 0.2 % of CoQ10 and intraperitoneal injection of minocycline (5 mg/kg/day)—separate or combined | R6/2 (HD) | ✓ | Prolonged life span; improved rotarod phenotype; reduced body weight loss (CoQ10 specific); attenuated gross brain atrophy and ventricular enlargement; attenuated neuronal atrophy; reduced aggregate formation (CoQ10 specific); attenuated the microglial response (minocycline specific) | Stack et al. 2006 [188] |
| CoQ10/creatine | Food supplemented with 1 % of CoQ10 and 2 % of creatine—separate or combined | R6/2 (HD) | ✓ | Improved rotarod performance; prolonged life span | Yang et al. 2009 [189] |
| CoQ10 | Food supplemented with 0.2 % of CoQ10 | R6/2 (HD) | × | No change in survival, body weight and rotarod performance; no change in rearing frequency and climbing performance; transient deleterious effects in the open field and grip strength; | Menalled et al. 2010 [190] |
| CoQ10 | Food supplemented with 0.6 % of CoQ10 | R6/2 (HD) | × | No change in survival, rotarod performance, grip strength performance, and climbing; transiently decreased body weight, locomotor activity, and rearing in R6/2 | Menalled et al. 2010 [190] |