Human COX7A2L regulates complex III biogenesis and promotes supercomplex organization remodeling without affecting mitochondrial bioenergetics

25 p.-7 fig.-1graph.abst.-5 fig.supl.

Detalles Bibliográficos
Autores: Lobo-Jarne, Teresa, Nyvltova, Eva, Pérez-Pérez, Rafael, Timón-Gómez, Alba, Molinié, Thibaut, Choi, Austin, Mourier, Arnaud, Fontanesi, Flavia, Ugalde, Cristina, Barrientos, Antoni
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2018
País:España
Institución:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/370514
Acceso en línea:http://hdl.handle.net/10261/370514
Access Level:acceso abierto
Palabra clave:Mitochondrial respiratory chain
Supercomplexes
Respirasomes
COX7A2L
COX7RP
Complex III
SCAFI
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spelling Human COX7A2L regulates complex III biogenesis and promotes supercomplex organization remodeling without affecting mitochondrial bioenergeticsLobo-Jarne, TeresaNyvltova, EvaPérez-Pérez, RafaelTimón-Gómez, AlbaMolinié, ThibautChoi, AustinMourier, ArnaudFontanesi, FlaviaUgalde, CristinaBarrientos, AntoniMitochondrial respiratory chainSupercomplexesRespirasomesCOX7A2LCOX7RPComplex IIISCAFI25 p.-7 fig.-1graph.abst.-5 fig.supl.The mitochondrial respiratory chain is organized in a dynamic set of supercomplexes (SCs). The COX7A2L protein is essential for mammalian SC III2+IV assembly. However, its function in respirasome (SCs I+III2+IVn) biogenesis remains controversial. To unambiguously determine the COX7A2L role, we generated COX7A2L-knockout (COX7A2L-KO) HEK293T and U87 cells. COX7A2L-KO cells lack SC III2+IV but have enhanced complex III steady-state levels, activity, and assembly rate, normal de novo complex IV biogenesis, and delayed respirasome formation. Nonetheless, the KOs have normal respirasome steady-state levels, and only larger structures (SCs I1-2+III2+IV2-n or megacomplexes) were undetected. Functional substrate-driven competition assays showed normal mitochondrial respiration in COX7A2L-KO cells in standard and nutritional-, environmental-, and oxidative-stress-challenging conditions. We conclude that COX7A2L establishes a regulatory checkpoint for the biogenesis of CIII2 and specific SCs, but the COX7A2L-dependent MRC remodeling is essential neither to maintain mitochondrial bioenergetics nor to cope with acute cellular stresses.This research was supported by NIH R01 Grants GM105781 (to A.B. and C.U.) and GM112179 (to A.B.), NIH R35 Grant GM118141 (to A.B.), MDA Grant MDA-381828 (to A.B.), AHA Development Grant 14SDG20040003 (to F.F.), and Instituto de Salud Carlos III-MINECO/European FEDER Funds Grants PI14-00209 and PI17-00048 (to C.U.). A.M. receives support from the AFM-Telethon (Trampoline Grant 19613) and ANR JCJC (ANR-16-CE14-0013).Peer reviewedCell PressNational Institutes of Health (US)Instituto de Salud Carlos IIIMinisterio de Economía y Competitividad (España)AFM-TéléthonLobo-Jarne, Teresa [0000-0002-1632-438X]Nyvltova, Eva [0000-0002-6607-2838]Timón-Gómez, Alba [0000-0001-9811-8557]Molinié, Thibaut [0000-0002-1774-671X]Choi, Austin [0000-0002-5476-4183]Mourier, Arnaud [0000-0002-5413-611X]Fontanesi, Flavia [0000-0003-0509-3835]Ugalde, Cristina [0000-0002-9742-1877]202420242018info:eu-repo/semantics/articlehttp://purl.org/coar/resource_type/c_6501Publisher's versioninfo:eu-repo/semantics/publishedVersionhttp://hdl.handle.net/10261/370514reponame:DIGITAL.CSIC. Repositorio Institucional del CSICinstname:Consejo Superior de Investigaciones Científicas (CSIC)Ingléshttps://doi.org/10.1016/j.celrep.2018.10.058Noinfo:eu-repo/semantics/openAccessoai:digital.csic.es:10261/3705142026-05-22T06:33:51Z
dc.title.none.fl_str_mv Human COX7A2L regulates complex III biogenesis and promotes supercomplex organization remodeling without affecting mitochondrial bioenergetics
title Human COX7A2L regulates complex III biogenesis and promotes supercomplex organization remodeling without affecting mitochondrial bioenergetics
spellingShingle Human COX7A2L regulates complex III biogenesis and promotes supercomplex organization remodeling without affecting mitochondrial bioenergetics
Lobo-Jarne, Teresa
Mitochondrial respiratory chain
Supercomplexes
Respirasomes
COX7A2L
COX7RP
Complex III
SCAFI
title_short Human COX7A2L regulates complex III biogenesis and promotes supercomplex organization remodeling without affecting mitochondrial bioenergetics
title_full Human COX7A2L regulates complex III biogenesis and promotes supercomplex organization remodeling without affecting mitochondrial bioenergetics
title_fullStr Human COX7A2L regulates complex III biogenesis and promotes supercomplex organization remodeling without affecting mitochondrial bioenergetics
title_full_unstemmed Human COX7A2L regulates complex III biogenesis and promotes supercomplex organization remodeling without affecting mitochondrial bioenergetics
title_sort Human COX7A2L regulates complex III biogenesis and promotes supercomplex organization remodeling without affecting mitochondrial bioenergetics
dc.creator.none.fl_str_mv Lobo-Jarne, Teresa
Nyvltova, Eva
Pérez-Pérez, Rafael
Timón-Gómez, Alba
Molinié, Thibaut
Choi, Austin
Mourier, Arnaud
Fontanesi, Flavia
Ugalde, Cristina
Barrientos, Antoni
author Lobo-Jarne, Teresa
author_facet Lobo-Jarne, Teresa
Nyvltova, Eva
Pérez-Pérez, Rafael
Timón-Gómez, Alba
Molinié, Thibaut
Choi, Austin
Mourier, Arnaud
Fontanesi, Flavia
Ugalde, Cristina
Barrientos, Antoni
author_role author
author2 Nyvltova, Eva
Pérez-Pérez, Rafael
Timón-Gómez, Alba
Molinié, Thibaut
Choi, Austin
Mourier, Arnaud
Fontanesi, Flavia
Ugalde, Cristina
Barrientos, Antoni
author2_role author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv National Institutes of Health (US)
Instituto de Salud Carlos III
Ministerio de Economía y Competitividad (España)
AFM-Téléthon
Lobo-Jarne, Teresa [0000-0002-1632-438X]
Nyvltova, Eva [0000-0002-6607-2838]
Timón-Gómez, Alba [0000-0001-9811-8557]
Molinié, Thibaut [0000-0002-1774-671X]
Choi, Austin [0000-0002-5476-4183]
Mourier, Arnaud [0000-0002-5413-611X]
Fontanesi, Flavia [0000-0003-0509-3835]
Ugalde, Cristina [0000-0002-9742-1877]
dc.subject.none.fl_str_mv Mitochondrial respiratory chain
Supercomplexes
Respirasomes
COX7A2L
COX7RP
Complex III
SCAFI
topic Mitochondrial respiratory chain
Supercomplexes
Respirasomes
COX7A2L
COX7RP
Complex III
SCAFI
description 25 p.-7 fig.-1graph.abst.-5 fig.supl.
publishDate 2018
dc.date.none.fl_str_mv 2018
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/10261/370514
url http://hdl.handle.net/10261/370514
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv https://doi.org/10.1016/j.celrep.2018.10.058
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 15.811543

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