Table 1.

Studies of effects of CoQ10 on healthy animals and animal models of neurodegenerative disease

Model	Dose	Effect	Ref
Healthy rats	200 mg/kg daily × 1–2 months	30%–40% increase in cerebral mitochondrial CoQ concentrations	Matthews2
Huntington’s models
Malonate	200 mg/kg daily × 1–2 months	Prevented depletion of ATP Attenuated striatal lesions With nicotinamide, prevented increase in lactate on MRS	Beal29
3-NP	200 mg/kg daily × 1–2 months	Attenuation of striatal lesions by 90%	Matthews2
N171–82Q mice	500 mg/kg/day	Diminished motor symptoms No effect on survival Effect augmented by remacimide	Ferrante45
R6/R2 and N171–82Q transgenic mice	CoQ10 400 mg/kg/day ± remacemide 14 mg/kg/day	Prolonged survival (additive) Delayed motor deficits, weight loss, cerebral atrophy, and neuronal intranuclear inclusions	Ferrante45
R6/R2 transgenic mice	1,000 to 20,000 mg/kg/day	Prolonged survival Improved motor performance Reduced weight loss Decrease in cerebral and striatal neuron atrophy, Htt aggregate accumulation	Smith8
R6/2 transgenic mice	CoQ10 (0.2%) and minocycline 5 mg/kg/day	Extended survival Improved motor performance Amerliorated weight loss and Htt aggregation (CoQ dominant effect)	Stack71
3-NP	CoQ10 (1%) and creatine (2%) × two weeks	Reduced striatal lesion volumes (additive)	Yang72
R6/2 transgenic mice	CoQ10 (1%) and creatine (2%) × 9–11 weeks	Improved motor performance (additive) Extended survival (additive)	Yang72
Parkinson’s models
MPTP	CoQ10 200 mg/kg/day × five weeks	Protected against: Striatal dopamine depletion Loss of TH-IR neurons in the SNpc	Beal27
MPTP	CoQ10 1600 mg/kg × 3.5 months	Protected against: Dopamine depletion Loss of TH-IR neurons in the SNpc Development of α-synuclein aggregates in SNpc	Cleren68
MPTP	CoQ10 (1%) and creatine (2%) × 5 weeks	Protected against: Dopamine depletion in the striatum (additive) Loss of tyrosine hydroxylase neurons in SNpc (additive) Lipid peroxidation α-synuclein aggregates in SNpc	Yang72
ALS models
G93 A SOD1 transgenic mouse	200 mg/kg daily from day 50 until end-stage disease	Significantly increased median survival by 4.4% (six days)	Matthews2
Alzheimer models
Aged APP, PS1, APP/PS1 transgenic mice	2,400 mg/kg/day × 60 days	Attenuated brain atrophy as assessed by MRI in vivo	Li74
Aged PS1 transgenic mouse	1,200 mg/day × 60 days	Partially reduced Aβ overproduction and intracellular cortical deposits Attenuation of elevated malondialdehyde levels and downregulated SOD	Yang73
TG19959 mice	0.4% in diet	Protected against plaques Protected against memory loss as assessed by Morris water maze task	Kipiani75

Abbreviations: mg, milligram; kg, kilogram; CoQ, coenzyme Q; CoQ10, coenzyme Q10; ATP, adenosine triphosphate; MRS, magnetic resonance spectroscopy; 3-NP, 3-nitroproprionic acid; Htt, huntingtin; MPTP, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine; TH-IR, tyrosine hydroxylase-immunoreactive; SNpc, substantia nigra pars compacta; SOD, superoxide dismutase; Aβ, β-amyloid; APP, amyloid precursor protein; PS1, presenilin 1; MRI, magnetic resonance imaging.