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Impaired PLP-dependent metabolism in brain samples from Huntington disease patients and transgenic R6/1 mice

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Issue date
2016-06
Author
Sorolla Bardají, Maria Alba
Rodríguez Colman, Maria José
Vall-llaura Espinosa, Núria
Vived Maza, Celia
Fernández Nogales, Marta
Lucas, José J.
Ferrer, Isidre
Cabiscol Català, Elisa
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Sorolla Bardají, Maria Alba; Rodríguez Colman, Maria José; Vall-llaura Espinosa, Núria; Vived Maza, Celia; Fernández Nogales, Marta; Lucas, José J.; ... Cabiscol Català, Elisa. (2016) . Impaired PLP-dependent metabolism in brain samples from Huntington disease patients and transgenic R6/1 mice. Metabolic Brain Disease, 2016, vol. 31, num. 3, p. 579-586. https://doi.org/10.1007/s11011-015-9777-7.
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Abstract
Oxidative stress has been described as important to Huntington disease (HD) progression. In a previous HD study, we identified several carbonylated proteins, including pyridoxal kinase and antiquitin, both of which are involved in the metabolism of pyridoxal 5A '-phosphate (PLP), the active form of vitamin B6. In the present study, pyridoxal kinase levels were quantified and showed to be decreased both in HD patients and a R6/1 mouse model, compared to control samples. A metabolomic analysis was used to analyze metabolites in brain samples of HD patients and R6/1 mice, compared to control samples using mass spectrometry. This technique allowed detection of increased concentrations of pyridoxal, the substrate of pyridoxal kinase. In addition, PLP, the product of the reaction, was decreased in striatum from R6/1 mice. Furthermore, glutamate and cystathionine, both substrates of PLP-dependent enzymes were increased in HD. This reinforces the hypothesis that PLP synthesis is impaired, and could explain some alterations observed in the disease. Together, these results identify PLP as a potential therapeutic agent.
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http://hdl.handle.net/10459.1/57916
DOI
https://doi.org/10.1007/s11011-015-9777-7
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Metabolic Brain Disease, 2016, vol. 31, num. 3, p. 579-586
European research projects
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