Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV

19 p.-8 fig.-1 tab.

Detalles Bibliográficos
Autores: Protasoni, Margherita, Pérez-Pérez, Rafael, Lobo-Jarne, Teresa, Harbour, Michael E., Ding, Shujing, Peñas, Ana, Diaz, Francisca, Moraes, Carlos T., Fearnley, Ian M., Zeviani, Massimo, Ugalde, Cristina, Fernandez-Vizarra, Erika
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2020
País:España
Institución:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/369046
Acceso en línea:http://hdl.handle.net/10261/369046
Access Level:acceso abierto
Palabra clave:Complex I
Complex III
Cytochrome b mutation
Mitochondrial respiratory chain assembly
Supercomplexes
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spelling Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IVProtasoni, MargheritaPérez-Pérez, RafaelLobo-Jarne, TeresaHarbour, Michael E.Ding, ShujingPeñas, AnaDiaz, FranciscaMoraes, Carlos T.Fearnley, Ian M.Zeviani, MassimoUgalde, CristinaFernandez-Vizarra, ErikaComplex IComplex IIICytochrome b mutationMitochondrial respiratory chain assemblySupercomplexes19 p.-8 fig.-1 tab.Mitochondrial respiratory chain (MRC) enzymes associate in supercomplexes (SCs) that are structurally interdependent. This may explain why defects in a single component often produce combined enzyme deficiencies in patients. A case in point is the alleged destabilization of complex I in the absence of complex III. To clarify the structural and functional relationships between complexes, we have used comprehensive proteomic, functional, and biogenetical approaches to analyze a MT‐CYB‐deficient human cell line. We show that the absence of complex III blocks complex I biogenesis by preventing the incorporation of the NADH module rather than decreasing its stability. In addition, complex IV subunits appeared sequestered within complex III subassemblies, leading to defective complex IV assembly as well. Therefore, we propose that complex III is central for MRC maturation and SC formation. Our results challenge the notion that SC biogenesis requires the pre‐formation of fully assembled individual complexes. In contrast, they support a cooperative‐assembly model in which the main role of complex III in SCs is to provide a structural and functional platform for the completion of overall MRC biogenesis.Our research was supported by the Core Grant from the Medical Research Council (Grant MC_UU_00015/5), ERC Advanced Grant FP7‐3222424 and NRJ‐Institut de France Grant (to M.Z.), Association Française contre les Myopathies (AFM) grant 16086 (to E.F‐V.), Instituto de Salud Carlos III‐MINECO/European ERDF‐ESF grant PI17‐00048, Comunidad Autónoma de Madrid/ERDF‐ESF grant P2018/BAA‐4403, and NIH‐RO1 grant GM105781 (to C.U.).Peer reviewedMedical Research Council (UK)European CommissionInstitut de FranceAssociation Française contre les MyopathiesInstituto de Salud Carlos IIIMinisterio de Economía y Competitividad (España)Comunidad de MadridProtasoni, Margherita [0000-0001-6557-461X]Lobo-Jarne, Teresa [0000-0002-1632-438X]Ding, Shujing [0009-0005-9320-7936]Peñas, Ana [0000-0003-2676-9953]Moraes, Carlos T. [0000-0002-8077-7092]Fearnley, Ian M. [0000-0002-8510-1219]Zeviani, Massimo [0000-0002-9067-5508]Ugalde, Cristina [0000-0002-9742-1877]Fernandez-Vizarra, Erika [0000-0002-2469-142X]202420242020info:eu-repo/semantics/articlehttp://purl.org/coar/resource_type/c_6501Publisher's versioninfo:eu-repo/semantics/publishedVersionhttp://hdl.handle.net/10261/369046reponame:DIGITAL.CSIC. Repositorio Institucional del CSICinstname:Consejo Superior de Investigaciones Científicas (CSIC)Inglés#PLACEHOLDER_PARENT_METADATA_VALUE##PLACEHOLDER_PARENT_METADATA_VALUE##PLACEHOLDER_PARENT_METADATA_VALUE#info:eu-repo/grantAgreement/EC/FP7/3222424P2018/BAA‐4403GM105781https://doi.org/10.15252/embj.2019102817Noinfo:eu-repo/semantics/openAccessoai:digital.csic.es:10261/3690462026-05-22T06:33:51Z
dc.title.none.fl_str_mv Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV
title Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV
spellingShingle Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV
Protasoni, Margherita
Complex I
Complex III
Cytochrome b mutation
Mitochondrial respiratory chain assembly
Supercomplexes
title_short Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV
title_full Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV
title_fullStr Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV
title_full_unstemmed Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV
title_sort Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV
dc.creator.none.fl_str_mv Protasoni, Margherita
Pérez-Pérez, Rafael
Lobo-Jarne, Teresa
Harbour, Michael E.
Ding, Shujing
Peñas, Ana
Diaz, Francisca
Moraes, Carlos T.
Fearnley, Ian M.
Zeviani, Massimo
Ugalde, Cristina
Fernandez-Vizarra, Erika
author Protasoni, Margherita
author_facet Protasoni, Margherita
Pérez-Pérez, Rafael
Lobo-Jarne, Teresa
Harbour, Michael E.
Ding, Shujing
Peñas, Ana
Diaz, Francisca
Moraes, Carlos T.
Fearnley, Ian M.
Zeviani, Massimo
Ugalde, Cristina
Fernandez-Vizarra, Erika
author_role author
author2 Pérez-Pérez, Rafael
Lobo-Jarne, Teresa
Harbour, Michael E.
Ding, Shujing
Peñas, Ana
Diaz, Francisca
Moraes, Carlos T.
Fearnley, Ian M.
Zeviani, Massimo
Ugalde, Cristina
Fernandez-Vizarra, Erika
author2_role author
author
author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Medical Research Council (UK)
European Commission
Institut de France
Association Française contre les Myopathies
Instituto de Salud Carlos III
Ministerio de Economía y Competitividad (España)
Comunidad de Madrid
Protasoni, Margherita [0000-0001-6557-461X]
Lobo-Jarne, Teresa [0000-0002-1632-438X]
Ding, Shujing [0009-0005-9320-7936]
Peñas, Ana [0000-0003-2676-9953]
Moraes, Carlos T. [0000-0002-8077-7092]
Fearnley, Ian M. [0000-0002-8510-1219]
Zeviani, Massimo [0000-0002-9067-5508]
Ugalde, Cristina [0000-0002-9742-1877]
Fernandez-Vizarra, Erika [0000-0002-2469-142X]
dc.subject.none.fl_str_mv Complex I
Complex III
Cytochrome b mutation
Mitochondrial respiratory chain assembly
Supercomplexes
topic Complex I
Complex III
Cytochrome b mutation
Mitochondrial respiratory chain assembly
Supercomplexes
description 19 p.-8 fig.-1 tab.
publishDate 2020
dc.date.none.fl_str_mv 2020
2024
2024
dc.type.none.fl_str_mv info:eu-repo/semantics/article
http://purl.org/coar/resource_type/c_6501
Publisher's version
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/10261/369046
url http://hdl.handle.net/10261/369046
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv #PLACEHOLDER_PARENT_METADATA_VALUE#
#PLACEHOLDER_PARENT_METADATA_VALUE#
#PLACEHOLDER_PARENT_METADATA_VALUE#
info:eu-repo/grantAgreement/EC/FP7/3222424
P2018/BAA‐4403
GM105781
https://doi.org/10.15252/embj.2019102817
No
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
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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