The University of Southampton
University of Southampton Institutional Repository

Huntingtin inclusion bodies are iron-dependent centers of oxidative events

Firdaus, Wance J.J., Wyttenbach, Andreas, Giuliano, Paola, Kretz-Remy, Carole, Currie, R. William and Arrigo, André-Patrick (2006) Huntingtin inclusion bodies are iron-dependent centers of oxidative events Febs Journal, 273, (23), pp. 5428-5441. (doi:10.1111/j.1742-4658.2006.05537.x).

Record type: Article

Abstract

Recently, we reported that the transient expression of huntingtin exon1 polypeptide containing polyglutamine tracts of various sizes (httEx1-polyQ) in cell models of Huntington disease generated an oxidative stress whose intensity was CAG repeat expansion-dependent. Here, we have analyzed the intracellular localization of the oxidative events generated by the httEx1-polyQ polypeptides. Analysis of live COS-7 cells as well as neuronal SK-N-SH and PC12 cells incubated with hydroethidine or dichlorofluorescein diacetate revealed oxidation of these probes at the level of the inclusion bodies formed by httEx1-polyQ polypeptides. The intensity and frequency of the oxidative events among the inclusions were CAG repeat expansion-dependent. Electron microscopic analysis of cell sections revealed the presence of oxidation-dependent morphologic alterations in the vicinity of httEx1-polyQ inclusion bodies. Moreover, a high level of oxidized proteins was recovered in partially purified inclusions. We also report that the iron chelator deferroxamine altered the structure, localization and oxidative potential of httEx1-polyQ inclusion bodies. Hence, despite the fact that the formation of inclusion bodies may represent a defense reaction of the cell to eliminate httEx1 mutant polypeptide, this phenomenon appears inherent to the generation of iron-dependent oxidative events that can be deleterious to the cell.

Full text not available from this repository.

More information

Published date: 1 December 2006
Keywords: HSPS, huntingtin, inclusion bodies, iron, ROS

Identifiers

Local EPrints ID: 56800
URI: http://eprints.soton.ac.uk/id/eprint/56800
ISSN: 1742-464X
PURE UUID: 969b584d-b265-4d0a-9f90-e398aace91a0

Catalogue record

Date deposited: 06 Aug 2008
Last modified: 17 Jul 2017 14:30

Export record

Altmetrics

Contributors

Author: Wance J.J. Firdaus
Author: Andreas Wyttenbach
Author: Paola Giuliano
Author: Carole Kretz-Remy
Author: R. William Currie
Author: André-Patrick Arrigo

University divisions

Download statistics

Downloads from ePrints over the past year. Other digital versions may also be available to download e.g. from the publisher's website.

View more statistics

Atom RSS 1.0 RSS 2.0

Contact ePrints Soton: eprints@soton.ac.uk

ePrints Soton supports OAI 2.0 with a base URL of http://eprints.soton.ac.uk/cgi/oai2

This repository has been built using EPrints software, developed at the University of Southampton, but available to everyone to use.

We use cookies to ensure that we give you the best experience on our website. If you continue without changing your settings, we will assume that you are happy to receive cookies on the University of Southampton website.

×