Mammalian frataxin directly enhances sulfur transfer of NFS1 persulfide to both ISCU and free thiols

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RECERCAT Home > Universitat de Barcelona > Química Inorgànica i Orgànica > Articles publicats en revistes (Química Inorgànica i Orgànica) > View document

To access the full text documents, please follow this link: http://hdl.handle.net/2445/67192

dc.contributor	Universitat de Barcelona
dc.contributor.author	Parent, Aubérie
dc.contributor.author	Elduque Busquets, Xavier
dc.contributor.author	Cornu, David
dc.contributor.author	Belot, Laura
dc.contributor.author	Le Caer, Jean-Pierre
dc.contributor.author	Grandas Sagarra, Anna
dc.contributor.author	Toledano, Michel B.
dc.contributor.author	D'Autréaux, Benoit
dc.date	2015-10-08T10:35:25Z
dc.date	2015-10-08T10:35:25Z
dc.date	2014-01-19
dc.date	2015-10-08T10:35:25Z
dc.identifier.citation	2041-1723
dc.identifier.citation	652080
dc.identifier.uri	http://hdl.handle.net/2445/67192
dc.format	12 p.
dc.format	application/pdf
dc.language.iso	eng
dc.publisher	Nature Publishing Group
dc.relation	Versió postprint del document publicat a: http://dx.doi.org/10.1038/ncomms6686
dc.relation	Nature Communications, 2014, vol. 6
dc.relation	http://dx.doi.org/10.1038/ncomms6686
dc.rights	(c) Parent et al., 2014
dc.rights	info:eu-repo/semantics/openAccess
dc.subject	Bioquímica
dc.subject	Malalties neurodegeneratives
dc.subject	Proteïnes
dc.subject	Biochemistry
dc.subject	Neurodegenerative diseases
dc.subject	Proteins
dc.title	Mammalian frataxin directly enhances sulfur transfer of NFS1 persulfide to both ISCU and free thiols
dc.type	info:eu-repo/semantics/article
dc.type	info:eu-repo/semantics/acceptedVersion
dc.description.abstract	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 com- pounds 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.

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