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Vascular dysfunction in a mouse model of Rett syndrome and effects of curcumin treatment.

التفاصيل البيبلوغرافية
العنوان: Vascular dysfunction in a mouse model of Rett syndrome and effects of curcumin treatment.
المؤلفون: Anna Panighini, Emiliano Duranti, Ferruccio Santini, Margherita Maffei, Tommaso Pizzorusso, Niccola Funel, Stefano Taddei, Nunzia Bernardini, Chiara Ippolito, Agostino Virdis, Mario Costa
المصدر: PLoS ONE, Vol 8, Iss 5, p e64863 (2013)
بيانات النشر: Public Library of Science (PLoS), 2013.
سنة النشر: 2013
المجموعة: LCC:Medicine
LCC:Science
مصطلحات موضوعية: Medicine, Science
الوصف: Mutations in the coding sequence of the X-linked gene MeCP2 (Methyl CpG-binding protein) are present in around 80% of patients with Rett Syndrome, a common cause of intellectual disability in female and to date without any effective pharmacological treatment. A relevant, and so far unexplored feature of RTT patients, is a marked reduction in peripheral circulation. To investigate the relationship between loss of MeCP2 and this clinical aspect, we used the MeCP2 null mouse model B6.129SF1-MeCP2tm1Jae for functional and pharmacological studies. Functional experiments were performed on isolated resistance mesenteric vessels, mounted on a pressurized myograph. Vessels from female MeCP2(+/-) mice show a reduced endothelium-dependent relaxation, due to a reduced Nitric Oxide (NO) availability secondary to an increased Reactive Oxygen Species (ROS) generation. Such functional aspects are associated with an intravascular increase in superoxide anion production, and a decreased vascular eNOS expression. These alterations are reversed by curcumin administration (5% (w/w) dietary curcumin for 21 days), which restores endothelial NO availability, decreases intravascular ROS production and normalizes vascular eNOS gene expression. In conclusion our findings highlight alterations in the vascular/endothelial system in the absence of a correct function of MeCP2, and uncover related cellular/molecular mechanisms that are rescued by an anti-oxidant treatment.
نوع الوثيقة: article
وصف الملف: electronic resource
اللغة: English
تدمد: 1932-6203
Relation: http://europepmc.org/articles/PMC3660336?pdf=render; https://doaj.org/toc/1932-6203
DOI: 10.1371/journal.pone.0064863
URL الوصول: https://doaj.org/article/66b1d478ce13446db2827ef5df0a2e7c
رقم الأكسشن: edsdoj.66b1d478ce13446db2827ef5df0a2e7c
قاعدة البيانات: Directory of Open Access Journals
الوصف
تدمد:19326203
DOI:10.1371/journal.pone.0064863