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| Content Provider | PubMed Central |
|---|---|
| Author | Felice, Claudio De Signorini, Cinzia Durand, Thierry Oger, Camille Guy, Alexandre Bultel-poncé, Valérie Galano, Jean-marie Lucia, Ciccoli Leoncini, Silvia Maurizio, D'esposito Filosa, Stefania Pecorelli, Alessandra Giuseppe, Valacchi Hayek, Joussef |
| Copyright Year | 2011 |
| Abstract | 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 F2-dihomo-isoprostanes (F2-dihomo-IsoPs), peroxidation products from adrenic acid (22:4 n-6), a known component of myelin, and tested the potential value of F2-dihomo-IsoPs as a novel disease marker and its relationship with clinical presentation and disease progression. F2-dihomo-IsoPs were determined by gas chromatography/negative-ion chemical ionization tandem mass spectrometry. Newly synthesized F2-dihomo-IsoP isomers [ent-7(RS)-F2t-dihomo-IsoP and 17-F2t-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)-F2t-dihomo-IsoP and 17-F2t-dihomo-IsoP. Average plasma F2-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. hese data indicate for the first time that quantification of F2-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 |
| Related Links | http://dx.doi.org/10.1194/jlr.p017798 |
| Ending Page | 2297 |
| Page Count | 11 |
| Starting Page | 2287 |
| File Format | |
| ISSN | 00222275 |
| e-ISSN | 15397262 |
| Journal | Journal of Lipid Research |
| Issue Number | 12 |
| Volume Number | 52 |
| Language | English |
| Publisher | The American Society for Biochemistry and Molecular Biology |
| Publisher Date | 2011-12-01 |
| Access Restriction | Open |
| Rights Holder | The American Society for Biochemistry and Molecular Biology |
| Subject Keyword | Cell Biology Biochemistry Endocrinology Research in Higher Education |
| Content Type | Text |
| Resource Type | Article |
| Subject | Cell Biology Biochemistry Endocrinology |
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