کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
6021945 | 1580656 | 2014 | 12 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Oxidative brain damage in Mecp2-mutant murine models of Rett syndrome
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کلمات کلیدی
non-protein-bound ironNPBIF4-neuroprostanesCRE4-HNEIsoPsCre-recombinaseF2-IsoPsRTTF2-isoprostanesPUFAsMecp24-hydroxy-2-nonenal - 4-هیدروکسی-2 غیرنالASDs - ASD هاBDNF - BDNF یا فاکتور نورونزایی مشتقشده از مغز PsV - PSVROS - ROSNeurodevelopmental disorder - اختلال نوروپاتولوژیautism spectrum disorders - اختلالات طیف اوتیسمAUs - استرالیاAdrenic acid - اسید آدرنیکArachidonic acid - اسید آراشیدونیکdocosahexaenoic acid - اسید داکوزاگزوائونیکPolyunsaturated fatty acids - اسید چرب اشباع نشدهIsoprostanes - ایزوپروتامین هاOxidative stress - تنش اکسیداتیوARA - در حال حاضرDHA - دوکوساهگزائنوئیک اسیدRett syndrome - سندروم رتBrain damage - ضربه مغزیBrain-derived neurotrophic factor - فاکتور نوروتروفی مشتق شده از مغزMurine models - مدل های مویPreserved speech variant - نوع سخنرانی حفظ شدهwild type - نوع وحشیarbitrary units - واحد دلخواهADA - وجود داردLipid peroxidation - پراکسیداسیون لیپیدReactive oxygen species - گونههای فعال اکسیژن
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
عصب شناسی
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
Rett syndrome (RTT) is a rare neurodevelopmental disorder affecting almost exclusively females, caused in the overwhelming majority of the cases by loss-of-function mutations in the gene encoding methyl-CpG binding protein 2 (MECP2). High circulating levels of oxidative stress (OS) markers in patients suggest the involvement of OS in the RTT pathogenesis. To investigate the occurrence of oxidative brain damage in Mecp2 mutant mouse models, several OS markers were evaluated in whole brains of Mecp2-null (pre-symptomatic, symptomatic, and rescued) and Mecp2-308 mutated (pre-symptomatic and symptomatic) mice, and compared to those of wild type littermates. Selected OS markers included non-protein-bound iron, isoprostanes (F2-isoprostanes, F4-neuroprostanes, F2-dihomo-isoprostanes) and 4-hydroxy-2-nonenal protein adducts. Our findings indicate that oxidative brain damage 1) occurs in both Mecp2-null (both â/y and stop/y) and Mecp2-308 (both 308/y males and 308/+ females) mouse models of RTT; 2) precedes the onset of symptoms in both Mecp2-null and Mecp2-308 models; and 3) is rescued by Mecp2 brain specific gene reactivation. Our data provide direct evidence of the link between Mecp2 deficiency, oxidative stress and RTT pathology, as demonstrated by the rescue of the brain oxidative homeostasis following brain-specifically Mecp2-reactivated mice. The present study indicates that oxidative brain damage is a previously unrecognized hallmark feature of murine RTT, and suggests that Mecp2 is involved in the protection of the brain from oxidative stress.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Neurobiology of Disease - Volume 68, August 2014, Pages 66-77
Journal: Neurobiology of Disease - Volume 68, August 2014, Pages 66-77
نویسندگان
Claudio De Felice, Floriana Della Ragione, Cinzia Signorini, Silvia Leoncini, Alessandra Pecorelli, Lucia Ciccoli, Francesco Scalabrì, Federico Marracino, Michele Madonna, Giuseppe Belmonte, Laura Ricceri, Bianca De Filippis, Giovanni Laviola,