2026-06-14T06:44:05Zhttps://repisalud.isciii.es/rest/oai/requestoai:repisalud.isciii.es:20.500.12105/65372024-10-31T11:40:12Zcom_20.500.12105_19604com_20.500.12105_2051col_20.500.12105_19605col_20.500.12105_19607
Repisalud
author
Quintana-Cabrera, Ruben
author
Quirin, Charlotte
author
Glytsou, Christina
author
Corrado, Mauro
author
Urbani, Andrea
author
Pellattiero, Anna
author
Calvo, Enrique
author
Vazquez, Jesus
author
Enriquez, Jose Antonio
author
Gerle, Christoph
author
Soriano, Maria Eugenia
author
Bernardi, Paolo
author
Scorrano, Luca
funder
Italian Association for Cancer Research
funder
Fondazione Umberto Veronesi
funder
Ministerio de Economía, Industria y Competitividad (España)
funder
Fondazione Telethon
funder
Unión Europea. Comisión Europea. European Research Council (ERC)
funder
Ministero dell Università e della Ricerca (Italia)
funder
Unión Europea. Comisión Europea
funder
Ministry of Education, Culture, Sports, Science, and Technology (Japón)
funder
Fundación ProCNIC
2018-10-26T07:59:26Z
2018-10-26T07:59:26Z
2018
Nat Commun. 2018; 9(1):3399
10.1038/s41467-018-05655-x
2041-1723
Nature Communications
30143614
http://hdl.handle.net/20.500.12105/6537
It is unclear how the mitochondrial fusion protein Optic atrophy 1 (OPA1), which inhibits cristae remodeling, protects from mitochondrial dysfunction. Here we identify the mitochondrial F1Fo-ATP synthase as the effector of OPA1 in mitochondrial protection. In OPA1 overexpressing cells, the loss of proton electrochemical gradient caused by respiratory chain complex III inhibition is blunted and this protection is abolished by the ATP synthase inhibitor oligomycin. Mechanistically, OPA1 and ATP synthase can interact, but recombinant OPA1 fails to promote oligomerization of purified ATP synthase reconstituted in liposomes, suggesting that OPA1 favors ATP synthase oligomerization and reversal activity by modulating cristae shape. When ATP synthase oligomers are genetically destabilized by silencing the key dimerization subunit e, OPA1 is no longer able to preserve mitochondrial function and cell viability upon complex III inhibition. Thus, OPA1 protects mitochondria from respiratory chain inhibition by stabilizing cristae shape and favoring ATP synthase oligomerization.
eng
PERMEABILITY TRANSITION PORE
COMPLEX III DEFICIENCY
CYTOCHROME-C RELEASE
OXIDATIVE-PHOSPHORYLATION
F1F0-ATP SYNTHASE
SUPRAMOLECULAR ORGANIZATION
LIVER-MITOCHONDRIA
INHIBITOR PROTEIN
INNER MEMBRANE
SUBUNIT-G
The cristae modulator Optic atrophy 1 requires mitochondrial ATP synthase oligomers to safeguard mitochondrial function
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