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Human and transgenic animal studies have implicated defective energy metabolism and oxidative stress in Huntington’s disease (HD), with evidence for deficiencies of respiratory chain activity and defective adenosine triphosphate (ATP) synthesis in brain and skeletal muscle.1,2⇓ Correlation of the muscle bioenergetic defect and CAG repeat length provides a link between this biochemical abnormality and the HD molecular defect.1 The role of calcium-mediated excitotoxicity and mitochondrial dysfunction is also supported by recent findings of a mitochondrial calcium handling defect.3
In the R6/2 HD transgenic mouse, 2% dietary creatine supplementation significantly improved survival, delayed motor progression, and slowed development of brain atrophy.4 Creatine has several potential neuroprotective effects, including buffering intracellular mitochondrial energy reserves, stabilizing intracellular calcium, and inhibiting activation of the mitochondrial permeability transition pore, which have all been linked to apoptotic and oxidative cell death. Two trials have been reported where patients with HD were given 5 g for 12 months5 and 3g for 2 months followed by 5 g for 2 months. …
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