The DOI matches its Crossref record; 9 of the 9 references it cites were found in a registry or an index.
Neuroprotective Effects of Creatine and Cyclocreatine in Animal Models of Huntington’s Disease
Open accessAbstract
The gene defect in Huntington’s disease (HD) may result in an impairment of energy metabolism. Malonate and 3-nitropropionic acid (3-NP) are inhibitors of succinate dehydrogenase that produce energy depletion and lesions that closely resemble those of HD. Oral supplementation with creatine or cyclocreatine, which are substrates for the enzyme creatine kinase, may increase phosphocreatine (PCr) or phosphocyclocreatine (PCCr) levels and ATP generation and thereby may exert neuroprotective effects. We found that oral supplementation with either creatine or cyclocreatine produced significant protection against malonate lesions, and that creatine but not cyclocreatine supplementation significantly protected against 3-NP neurotoxicity. Creatine and cyclocreatine increased brain concentrations of PCr and PCCr, respectively, and creatine protected against depletions of PCr and ATP produced by 3-NP. Creatine supplementation protected against 3-NP induced increases in striatal lactate concentrations in vivo as assessed by 1 H magnetic resonance spectroscopy. Creatine and cyclocreatine protected against malonate-induced increases in the conversion of salicylate to 2,3- and 2,5-dihydroxybenzoic acid, biochemical markers of hydroxyl radical generation. Creatine administration protected against 3-NP-induced increases in 3-nitrotyrosine concentrations, a marker of peroxynitrite-mediated oxidative injury. Oral supplementation with creatine or cyclocreatine results in neuroprotective effects in vivo, which may represent a novel therapeutic strategy for HD and other neurodegenerative diseases.
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“Key words: creatine; ATP; oxidative injury; 3-nitrotyrosine; 3-nitropropionic acid; Huntington There is substantial evidence that impairment of energy production may play a role in the pathogenesis of neurodegenerative diseases (Albin and Greenamyre, 1992; Beal, 1992).”
BackgroundCreatine deficiency in the brain: A new, treatable inborn error of metabolism
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Metadata
- Authors
- Matthews, R. T., Yang, L., Jenkins, B. G., Ferrante, R. J., Rosen, B. R., Kaddurah-Daouk, R., & Beal, M. F.
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 1998-01-01
- Volume
- 18
- Issue
- 1
- Pages
- 156-163
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- OpenAlex
- W2125591463
- Cited by
- 430
- References
- 9
- FWCI
- 11.22
- Type
- journal article
- Language
- en
- Open access
- hybrid
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