Mitochondrial complex I plays an essential role in human respirasome assembly

22 p.-7 fig.

Detalhes bibliográficos
Autores: Moreno-Lastres, David, Fontanesi, Flavia, García-Consuegra, Inés, Martín, Miguel Ángel, Arenas, Joaquín, Barrientos, Antoni, Ugalde, Cristina
Formato: artículo
Estado:Versión aceptada para publicación
Fecha de publicación:2012
País:España
Recursos:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/370395
Acesso em linha:http://hdl.handle.net/10261/370395
Access Level:acceso abierto
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spelling Mitochondrial complex I plays an essential role in human respirasome assemblyMoreno-Lastres, DavidFontanesi, FlaviaGarcía-Consuegra, InésMartín, Miguel ÁngelArenas, JoaquínBarrientos, AntoniUgalde, Cristina22 p.-7 fig.The biogenesis and function of the mitochondrial respiratory chain (RC) involve the organization of RC enzyme complexes in supercomplexes or respirasomes through an unknown biosynthetic process. This leads to structural interdependences between RC complexes, which are highly relevant from biological and biomedical perspectives, because RC defects often lead to severe neuromuscular disorders. We show that in human cells, respirasome biogenesis involves a complex I assembly intermediate acting as a scaffold for the combined incorporation of complexes III and IV subunits, rather than originating from the association of preassembled individual holoenzymes. The process ends with the incorporation of complex I NADH dehydrogenase catalytic module, which leads to the respirasome activation. While complexes III and IV assemble either as free holoenzymes or by incorporation of free subunits into supercomplexes, the respirasomes constitute the structural units where complex I is assembled and activated, thus explaining the significance of the respirasomes for RC function.Our study was supported by FIB Hospital Universitario 12 de Octubre and Instituto de Salud Carlos III to C.U. (grant numbers PI08-0021 and PI11-00182), by the Muscular Dystrophy Association to F.F. (grant number 158547) and A.B. (grant number 186025), and by National Institutes of Health (NIH) grant GM071775A to A.B.Peer reviewedCell PressInstituto de Investigación Hospital 12 de OctubreInstituto de Salud Carlos IIIMuscular Dystrophy Association (US)National Institutes of Health (US)Fontanesi, Flavia [0000-0003-0509-3835]García-Consuegra, Inés [0000-0003-1717-7446]Arenas, Joaquín [0000-0002-2877-5049]Ugalde, Cristina [0000-0002-9742-1877]202420242012info:eu-repo/semantics/articlehttp://purl.org/coar/resource_type/c_6501Postprintinfo:eu-repo/semantics/acceptedVersionhttp://hdl.handle.net/10261/370395reponame:DIGITAL.CSIC. Repositorio Institucional del CSICinstname:Consejo Superior de Investigaciones Científicas (CSIC)Ingléshttps://doi.org/10.1016/j.cmet.2012.01.015Noinfo:eu-repo/semantics/openAccessoai:digital.csic.es:10261/3703952026-05-22T06:33:51Z
dc.title.none.fl_str_mv Mitochondrial complex I plays an essential role in human respirasome assembly
title Mitochondrial complex I plays an essential role in human respirasome assembly
spellingShingle Mitochondrial complex I plays an essential role in human respirasome assembly
Moreno-Lastres, David
title_short Mitochondrial complex I plays an essential role in human respirasome assembly
title_full Mitochondrial complex I plays an essential role in human respirasome assembly
title_fullStr Mitochondrial complex I plays an essential role in human respirasome assembly
title_full_unstemmed Mitochondrial complex I plays an essential role in human respirasome assembly
title_sort Mitochondrial complex I plays an essential role in human respirasome assembly
dc.creator.none.fl_str_mv Moreno-Lastres, David
Fontanesi, Flavia
García-Consuegra, Inés
Martín, Miguel Ángel
Arenas, Joaquín
Barrientos, Antoni
Ugalde, Cristina
author Moreno-Lastres, David
author_facet Moreno-Lastres, David
Fontanesi, Flavia
García-Consuegra, Inés
Martín, Miguel Ángel
Arenas, Joaquín
Barrientos, Antoni
Ugalde, Cristina
author_role author
author2 Fontanesi, Flavia
García-Consuegra, Inés
Martín, Miguel Ángel
Arenas, Joaquín
Barrientos, Antoni
Ugalde, Cristina
author2_role author
author
author
author
author
author
dc.contributor.none.fl_str_mv Instituto de Investigación Hospital 12 de Octubre
Instituto de Salud Carlos III
Muscular Dystrophy Association (US)
National Institutes of Health (US)
Fontanesi, Flavia [0000-0003-0509-3835]
García-Consuegra, Inés [0000-0003-1717-7446]
Arenas, Joaquín [0000-0002-2877-5049]
Ugalde, Cristina [0000-0002-9742-1877]
description 22 p.-7 fig.
publishDate 2012
dc.date.none.fl_str_mv 2012
2024
2024
dc.type.none.fl_str_mv info:eu-repo/semantics/article
http://purl.org/coar/resource_type/c_6501
Postprint
info:eu-repo/semantics/acceptedVersion
format article
status_str acceptedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/10261/370395
url http://hdl.handle.net/10261/370395
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv https://doi.org/10.1016/j.cmet.2012.01.015
No
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv Cell Press
publisher.none.fl_str_mv Cell Press
dc.source.none.fl_str_mv reponame:DIGITAL.CSIC. Repositorio Institucional del CSIC
instname:Consejo Superior de Investigaciones Científicas (CSIC)
instname_str Consejo Superior de Investigaciones Científicas (CSIC)
reponame_str DIGITAL.CSIC. Repositorio Institucional del CSIC
collection DIGITAL.CSIC. Repositorio Institucional del CSIC
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15.812429

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