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Title: Impaired PLP-dependent metabolism in brain samples from Huntington disease patients and transgenic R6/1 mice
Author: Sorolla, M. Alba
Rodríguez-Colman, María José
Vall-Llaura, Núria
Vived, Celia
Fernández-Nogales, Marta
Lucas, José J.
Ferrer, Isidro (Ferrer Abizanda)
Cabiscol, Elisa
Keywords: Escorça cerebral
Corea de Huntington
Estrès oxidatiu
Cerebral cortex
Huntington's chorea
Oxidative stress
Issue Date: Jun-2016
Publisher: Springer-Verlag Germany
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 5´-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.
Note: Versió postprint del document publicat a:
It is part of: Metabolic Brain Disease, 2016, vol. 31, num. 3, p. 579-586
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ISSN: 0885-7490
Appears in Collections:Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
Articles publicats en revistes (Patologia i Terapèutica Experimental)

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