| http://purl.uniprot.org/citations/25597503 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/25597503 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/25597503 | http://www.w3.org/2000/01/rdf-schema#comment | "Friedreich's ataxia is a severe neurodegenerative disease caused by the decreased expression of frataxin, a mitochondrial protein that stimulates iron-sulfur (Fe-S) cluster biogenesis. In mammals, the primary steps of Fe-S cluster assembly are performed by the NFS1-ISD11-ISCU complex via the formation of a persulfide intermediate on NFS1. Here we show that frataxin modulates the reactivity of NFS1 persulfide with thiols. We use maleimide-peptide compounds along with mass spectrometry to probe cysteine-persulfide in NFS1 and ISCU. Our data reveal that in the presence of ISCU, frataxin enhances the rate of two similar reactions on NFS1 persulfide: sulfur transfer to ISCU leading to the accumulation of a persulfide on the cysteine C104 of ISCU, and sulfur transfer to small thiols such as DTT, L-cysteine and GSH leading to persulfuration of these thiols and ultimately sulfide release. These data raise important questions on the physiological mechanism of Fe-S cluster assembly and point to a unique function of frataxin as an enhancer of sulfur transfer within the NFS1-ISD11-ISCU complex."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.org/dc/terms/identifier | "doi:10.1038/ncomms6686"xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.org/dc/terms/identifier | "doi:10.1038/ncomms6686"xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Toledano M.B."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Toledano M.B."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Parent A."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Parent A."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Le Caer J.P."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Le Caer J.P."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Cornu D."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Cornu D."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "D'Autreaux B."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "D'Autreaux B."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Belot L."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Belot L."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Elduque X."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Elduque X."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Grandas A."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/author | "Grandas A."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/date | "2015"xsd:gYear |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/date | "2015"xsd:gYear |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/name | "Nat. Commun."xsd:string |
| http://purl.uniprot.org/citations/25597503 | http://purl.uniprot.org/core/name | "Nat. Commun."xsd:string |