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http://hdl.handle.net/2445/140541
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DC Field | Value | Language |
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dc.contributor.author | Gervason, Sylvain | - |
dc.contributor.author | Larkem, Djabir | - |
dc.contributor.author | Mansour, Amir Ben | - |
dc.contributor.author | Botzanowski, Thomas | - |
dc.contributor.author | Müller, Christina S. | - |
dc.contributor.author | Pecqueur, Ludovic | - |
dc.contributor.author | Le Pavec, Gwenaelle | - |
dc.contributor.author | Delaunay-Moisan, Agnès | - |
dc.contributor.author | Brun Cubero, Omar | - |
dc.contributor.author | Agramunt, Jordi | - |
dc.contributor.author | Grandas Sagarra, Anna | - |
dc.contributor.author | Fontecave, Marc | - |
dc.contributor.author | Schünemann, Volker | - |
dc.contributor.author | Cianférani, Sarah | - |
dc.contributor.author | Sizun, Christina | - |
dc.contributor.author | Toledano, Michel B. | - |
dc.contributor.author | D'Autréaux, Benoit | - |
dc.date.accessioned | 2019-09-19T14:47:35Z | - |
dc.date.available | 2019-09-19T14:47:35Z | - |
dc.date.issued | 2019 | - |
dc.identifier.issn | 2041-1723 | - |
dc.identifier.uri | http://hdl.handle.net/2445/140541 | - |
dc.description.abstract | Iron-sulfur (Fe-S) clusters are essential protein cofactors whose biosynthetic defects lead to severe diseases among which is Friedreich's ataxia caused by impaired expression of frataxin (FXN). Fe-S clusters are biosynthesized on the scaffold protein ISCU, with cysteine desulfurase NFS1 providing sulfur as persulfide and ferredoxin FDX2 supplying electrons, in a process stimulated by FXN but not clearly understood. Here, we report the breakdown of this process, made possible by removing a zinc ion in ISCU that hinders iron insertion and promotes non-physiological Fe-S cluster synthesis from free sulfide in vitro. By binding zinc-free ISCU, iron drives persulfide uptake from NFS1 and allows persulfide reduction into sulfide by FDX2, thereby coordinating sulfide production with its availability to generate Fe-S clusters. FXN stimulates the whole process by accelerating persulfide transfer. We propose that this reconstitution recapitulates physiological conditions which provides a model for Fe-S cluster biosynthesis, clarifies the roles of FDX2 and FXN and may help develop Friedreich's ataxia therapies. | - |
dc.format.extent | 13 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | Nature Publishing Group | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.1038/s41467-019-11470-9 | - |
dc.relation.ispartof | Nature Communications, 2019, vol. 10, p. 3566 | - |
dc.relation.uri | https://doi.org/10.1038/s41467-019-11470-9 | - |
dc.rights | cc-by (c) Gervason, Sylvain et al., 2019 | - |
dc.rights.uri | http://creativecommons.org/licenses/by/3.0/es | - |
dc.source | Articles publicats en revistes (Química Inorgànica i Orgànica) | - |
dc.subject.classification | Malalties neurodegeneratives | - |
dc.subject.classification | Bioquímica | - |
dc.subject.classification | Biosíntesi | - |
dc.subject.classification | Proteïnes | - |
dc.subject.other | Neurodegenerative Diseases | - |
dc.subject.other | Biochemistry | - |
dc.subject.other | Biosynthesis | - |
dc.subject.other | Proteins | - |
dc.title | Physiologically relevant reconstitution of iron-sulfur cluster biosynthesis uncovers persulfide- processing functions of ferredoxin-2 and frataxin | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.identifier.idgrec | 691256 | - |
dc.date.updated | 2019-09-19T14:47:36Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
dc.identifier.pmid | 31395877 | - |
Appears in Collections: | Articles publicats en revistes (Química Inorgànica i Orgànica) |
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