title: Reactive oxygen species are involved in ferroportin degradation induced by ceruloplasmin mutant Arg701Trp.
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.
authors.affiliation: Department of Biology, University Roma Tre, Rome, Italy.
authors.forename: Tiziana
authors.initials: T
authors.lastname: Persichini
authors.forename: Giovanni
authors.initials: G
authors.lastname: De Francesco
authors.forename: Caterina
authors.initials: C
authors.lastname: Capone
authors.forename: Antimo
authors.initials: A
authors.lastname: Cutone
authors.forename: Maria Carmela Bonaccorsi
authors.initials: MC
authors.lastname: di Patti
authors.forename: Marco
authors.initials: M
authors.lastname: Colasanti
authors.forename: Giovanni
authors.initials: G
authors.lastname: Musci
mesh_terms: D000818:Animals; D001120:Arginine / Q000737:chemistry*; D027682:Cation Transport Proteins / Q000378:metabolism*; D045744:Cell Line, Tumor; D002570:Ceruloplasmin / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D008856:Microscopy, Fluorescence; D009154:Mutation; D059748:Proteolysis; D051381:Rats; D017382:Reactive Oxygen Species / Q000378:metabolism*; D014364:Tryptophan / Q000737:chemistry*; D000098742:Ferroportin
chemical_list: D027682:Cation Transport Proteins; D017382:Reactive Oxygen Species; D000098742:Ferroportin; D014364:Tryptophan; D001120:Arginine; D002570:Ceruloplasmin