Redox Imbalance and Morphological Changes in Skin Fibroblasts in Typical Rett Syndrome
المؤلفون المشاركون
Paccagnini, Eugenio
Durand, Thierry
Gentile, Mariangela
Galano, Jean-Marie
Meloni, Ilaria
Ariani, Francesca
Mari, Francesca
Amabile, Sonia
Belmonte, Giuseppe
Renieri, Alessandra
Hayek, Joussef
Pecorelli, Alessandra
Leoncini, Silvia
Zollo, Gloria
Signorini, Cinzia
Ciccoli, Lucia
De Felice, Claudio
Giuseppe, Valacchi
المصدر
Oxidative Medicine and Cellular Longevity
العدد
المجلد 2014، العدد 2014 (31 ديسمبر/كانون الأول 2014)، ص ص. 1-10، 10ص.
الناشر
Hindawi Publishing Corporation
تاريخ النشر
2014-05-29
دولة النشر
مصر
عدد الصفحات
10
التخصصات الرئيسية
الملخص EN
Evidence of oxidative stress has been reported in the blood of patients with Rett syndrome (RTT), a neurodevelopmental disorder mainly caused by mutations in the gene encoding the Methyl-CpG-binding protein 2.
Little is known regarding the redox status in RTT cellular systems and its relationship with the morphological phenotype.
In RTT patients (n = 16) we investigated four different oxidative stress markers, F2-Isoprostanes (F2-IsoPs), F4-Neuroprostanes (F4-NeuroPs), nonprotein bound iron (NPBI), and (4-HNE PAs), and glutathione in one of the most accessible cells, that is, skin fibroblasts, and searched for possible changes in cellular/intracellular structure and qualitative modifications of synthesized collagen.
Significantly increased F4-NeuroPs (12-folds), F2-IsoPs (7.5-folds) NPBI (2.3-folds), 4-HNE PAs (1.48-folds), and GSSG (1.44-folds) were detected, with significantly decreased GSH (−43.6%) and GSH/GSSG ratio (−3.05 folds).
A marked dilation of the rough endoplasmic reticulum cisternae, associated with several cytoplasmic multilamellar bodies, was detectable in RTT fibroblasts.
Colocalization of collagen I and collagen III, as well as the percentage of type I collagen as derived by semiquantitative immunofluorescence staining analyses, appears to be significantly reduced in RTT cells.
Our findings indicate the presence of a redox imbalance and previously unrecognized morphological skin fibroblast abnormalities in RTT patients.
نمط استشهاد جمعية علماء النفس الأمريكية (APA)
Signorini, Cinzia& Leoncini, Silvia& De Felice, Claudio& Pecorelli, Alessandra& Meloni, Ilaria& Ariani, Francesca…[et al.]. 2014. Redox Imbalance and Morphological Changes in Skin Fibroblasts in Typical Rett Syndrome. Oxidative Medicine and Cellular Longevity،Vol. 2014, no. 2014, pp.1-10.
https://search.emarefa.net/detail/BIM-1046957
نمط استشهاد الجمعية الأمريكية للغات الحديثة (MLA)
Signorini, Cinzia…[et al.]. Redox Imbalance and Morphological Changes in Skin Fibroblasts in Typical Rett Syndrome. Oxidative Medicine and Cellular Longevity No. 2014 (Dec. 2014), pp.1-10.
https://search.emarefa.net/detail/BIM-1046957
نمط استشهاد الجمعية الطبية الأمريكية (AMA)
Signorini, Cinzia& Leoncini, Silvia& De Felice, Claudio& Pecorelli, Alessandra& Meloni, Ilaria& Ariani, Francesca…[et al.]. Redox Imbalance and Morphological Changes in Skin Fibroblasts in Typical Rett Syndrome. Oxidative Medicine and Cellular Longevity. 2014. Vol. 2014, no. 2014, pp.1-10.
https://search.emarefa.net/detail/BIM-1046957
نوع البيانات
مقالات
لغة النص
الإنجليزية
الملاحظات
Includes bibliographical references
رقم السجل
BIM-1046957
قاعدة معامل التأثير والاستشهادات المرجعية العربي "ارسيف Arcif"
أضخم قاعدة بيانات عربية للاستشهادات المرجعية للمجلات العلمية المحكمة الصادرة في العالم العربي
تقوم هذه الخدمة بالتحقق من التشابه أو الانتحال في الأبحاث والمقالات العلمية والأطروحات الجامعية والكتب والأبحاث باللغة العربية، وتحديد درجة التشابه أو أصالة الأعمال البحثية وحماية ملكيتها الفكرية. تعرف اكثر