Journal
JOURNAL OF LIPID RESEARCH
Volume 52, Issue 12, Pages 2287-2297Publisher
ELSEVIER
DOI: 10.1194/jlr.P017798
Keywords
methyl-CpG binding protein 2; adrenic acid; myelin damage; autism spectrum disorders
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Funding
- Nuovi approcci terapeutici nella sindrome di Rett, Siena, Italy [Orphan_0108]
- University of Montpellier I
- INRA [AlimH-2008-2010]
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Oxidative damage has been reported in Rett syndrome (RTT), a pervasive developmental disorder caused in up to 95% of cases by mutations in the X-linked methyl-CpG binding protein 2 gene. Herein, we have synthesized F-2-dihomo-isoprostanes (F-2-dihomo-IsoPs), peroxidation products from adrenic acid (22:4 n-6), a known component of myelin, and tested the potential value of F-2-dihomo-IsoPs as a novel disease marker and its relationship with clinical presentation and disease progression. F-2-dihomo-IsoPs were determined by gas chromatography/negative-ion chemical ionization tandem mass spectrometry. Newly synthesized F-2-dihomo-IsoP isomers [ent-7(RS)-F-2t-dihomo-IsoP and 17-F-2t-dihomo-IsoP] were used as reference standards. The measured ions were the product ions at m/z 327 derived from the [M-181](-) precursor ions (m/z 597) produced from both the derivatized ent-7(RS)-F-2t-dihomo-IsoP and 17-F-2t-dihomo-IsoP. Average plasma F-2-dihomo-IsoP levels in RTT were about one order of magnitude higher than those in healthy controls, being higher in typical RTT as compared with RTT variants, with a remarkable increase of about two orders of magnitude in patients at the earliest stage of the disease followed by a steady decrease during the natural clinical progression. These data indicate for the first time that quantification of F-2-dihomo-IsoPs in plasma represents an early marker of the disease and may provide a better understanding of the pathogenic mechanisms behind the neurological regression in patients with RTT.-De Felice, C., C. Signorini, T. Durand, C. Oger, A. Guy, V. Bultel-Ponce, J-M. Galano, L. Ciccoli, S. Leoncini, M. D'Esposito, S. Filosa, A. Pecorelli, G. Valacchi, and J. Hayek. F-2-dihomo-isoprostanes as potential early biomarkers of lipid oxidative damage in Rett syndrome. J. Lipid Res. 2011. 52: 2287-2297.
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