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dc.contributor.authorGonzález-Cabo, Pilar-
dc.contributor.authorBolinches-Amorós, Arantxa-
dc.contributor.authorCabello, Juan-
dc.contributor.authorRos, Sheila-
dc.contributor.authorMoreno, Sergio-
dc.contributor.authorPalau Martínez, Francesc-
dc.contributor.authorVázquez-Manrique, Rafael P.-
dc.identifier.citationJournal of Biological Chemistry 286(24): 21304-21314 (2011)es_ES
dc.description11 páginas, 6 figuras, 2 tablas.-- Free via Open Access: OA.-- PMID:21464130[PubMed] PMCID:PMC3122190es_ES
dc.description.abstractX-linked sideroblastic anemia with ataxia (XLSA/A) is a rare inherited disorder characterized by mild anemia and ataxia. XLSA/A is caused by mutations in the ABCB7 gene, which encodes a member of the ATP-binding cassette transporter family. Studies in yeast, mammalian cells, and mice have shown that ABCB7 functions in the transport of iron-sulfur (Fe-S) clusters into the cytoplasm. To further investigate the mechanism of this disease, we have identified and characterized the Caenorhabditis elegans homologue of the ABCB7 gene, abtm-1. We have studied the function of abtm-1 using mutants and RNAi. abtm-1-depleted animals produce arrested embryos that have morphogenetic defects and unusual premature, putative apoptotic events. abtm-1(RNAi) animals also show accumulation of ferric iron and increased oxidative stress. Despite the increased level of oxidative stress in abtm-1(RNAi) animals, they have an increased life span. We observed accumulation of DAF-16/FOXO in the nuclei of affected animals and elevation of the expression of SOD-3, a well established target of DAF-16, which may explain the increased life span extension of these animals. abtm-1 is strongly expressed in tissues with a high energy demand, and abtm-1(RNAi) animals have phenotypes that reflect the need for abtm-1 in these tissues. Finally, we show that reducing the function of other genes involved in Fe-S cluster production produces similar phenotypic consequences to abtm-1 loss of function. Therefore, ablation of abtm-1 in C. elegans provides a model in which to investigate the mechanism underlying XLSA/A.es_ES
dc.description.sponsorshipThis work was supported by The Spanish Ministry of Education and Science (grants SAF2006-01147, BFU2008-01808 and Consolider CSD2007-00015), Generalitat Valenciana, Junta de Castilla y León (grants CSI03A08 and Grupo de Excelencia GR 265), The Welcome Trust and MRC. The CIBERER is an initiative of the Instituto de Salud Carlos III.es_ES
dc.publisherAmerican Society for Biochemistry and Molecular Biologyes_ES
dc.titleDisruption of the ATP-binding cassette B7 (ABTM-1/ABCB7) induces oxidative stress and premature cell death in Caenorhabditis eleganses_ES
dc.description.peerreviewedPeer reviewedes_ES
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