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HD Lighthouse Contributing Editor's Comment: Creatine may provide more protection for mice than humans because mice exercise more. Mice don't have comfortable chairs and TVs. Any HD therapy should start as early as possible. The following creatine study suggests that even therapy started in late stage HD may be of benefit. --Jerry
HD Research
"The research in our laboratory investigates fundamental mechanisms of cell death in neurodegenerative diseases. We have principally focused on the role of mitochondrial dysfunction and oxidative damage in neurodegenerative diseases" --M. Flint Beal, M.D., Professor of Neuroscience, Cornell University. Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice.While there have been enormous strides in the understanding of Huntington's disease (HD) pathogenesis, treatment to slow or prevent disease progression remains elusive. We previously reported that dietary creatine supplementation significantly improves the clinical and neuropathological phenotype in transgenic HD mice lines starting at weaning, before clinical symptoms appear. We now report that creatine administration started after onset of clinical symptoms significantly extends survival in the R6/2 transgenic mouse model of HD. Creatine treatment started at 6, 8, and 10 weeks of age, analogous to early, middle, and late stages of human HD, significantly extended survival at both the 6- and 8-week starting points. Significantly improved motor performance was present in both the 6- and 8-week treatment paradigms, while reduced body weight loss was only observed in creatine-supplemented R6/2 mice started at 6 weeks. Neuropathological sequelae of gross brain and neuronal atrophy and huntingtin aggregates were delayed in creatine-treated R6/2 mice started at 6 weeks. We show significantly reduced brain levels of both creatine and ATP in R6/2 mice, consistent with a bioenergetic defect. Oral creatine supplementation significantly increased brain concentrations of creatine and ATP to wild-type control levels, exerting a neuroprotective effect. These findings have important therapeutic implications, suggesting that creatine therapy initiated after diagnosis may provide significant clinical benefits to HD patients. Source: J Neurochem. 2003 Jun;85(6):1359-1367, Dedeoglu A, et al.
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Exercise in mice models have been shown to delay the onset of HD. Exercise also improves the quality of life in many areas.
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