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Redox Imbalance and Morphological Changes in Skin Fibroblasts in Typical Rett Syndrome
Joint Authors
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
Source
Oxidative Medicine and Cellular Longevity
Issue
Vol. 2014, Issue 2014 (31 Dec. 2014), pp.1-10, 10 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2014-05-29
Country of Publication
Egypt
No. of Pages
10
Main Subjects
Abstract 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.
American Psychological Association (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
Modern Language Association (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
American Medical Association (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
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1046957