The ceruloplasmin mutant R701W, that causes a dramatic phenotype in the young heterozygous patient carrying this mutation, has been shown to have profound effects also in cell culture models. Here we show that Golgi rearrangement and degradation of the iron exporter ferroportin, that follow transfection of cells with this mutant, are accompanied by the massive production of reactive oxygen species (ROS) in the cell. Scavenging ROS production with different antioxidants, including reduced glutathione and zinc, restores Golgi morphology and rescues ferroportin on the cell membrane. (C) 2012 Elsevier Ltd. All rights reserved.

Reactive oxygen species are involved in ferroportin degradation induced by ceruloplasmin mutant Arg701Trp / Tiziana, Persichini; DE FRANCESCO, Giovanni; Caterina, Capone; Cutone, Antimo; BONACCORSI DI PATTI, Maria Carmela; Marco, Colasanti; Giovanni, Musci. - In: NEUROCHEMISTRY INTERNATIONAL. - ISSN 0197-0186. - 60:4(2012), pp. 360-364. [10.1016/j.neuint.2012.01.010]

Reactive oxygen species are involved in ferroportin degradation induced by ceruloplasmin mutant Arg701Trp

DE FRANCESCO, GIOVANNI;CUTONE, ANTIMO;BONACCORSI DI PATTI, Maria Carmela;
2012

Abstract

The ceruloplasmin mutant R701W, that causes a dramatic phenotype in the young heterozygous patient carrying this mutation, has been shown to have profound effects also in cell culture models. Here we show that Golgi rearrangement and degradation of the iron exporter ferroportin, that follow transfection of cells with this mutant, are accompanied by the massive production of reactive oxygen species (ROS) in the cell. Scavenging ROS production with different antioxidants, including reduced glutathione and zinc, restores Golgi morphology and rescues ferroportin on the cell membrane. (C) 2012 Elsevier Ltd. All rights reserved.
2012
aceruloplasminemia; astrocytes; ceruloplasmin; golgi; iron; oxidative stress
01 Pubblicazione su rivista::01a Articolo in rivista
Reactive oxygen species are involved in ferroportin degradation induced by ceruloplasmin mutant Arg701Trp / Tiziana, Persichini; DE FRANCESCO, Giovanni; Caterina, Capone; Cutone, Antimo; BONACCORSI DI PATTI, Maria Carmela; Marco, Colasanti; Giovanni, Musci. - In: NEUROCHEMISTRY INTERNATIONAL. - ISSN 0197-0186. - 60:4(2012), pp. 360-364. [10.1016/j.neuint.2012.01.010]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/438247
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