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Article

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

The EMBO journal - 3 Feb 2020

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, Fernández-Vizarra Erika

Abstract excerpt

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...

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Identifiers

PMID: 31912925
DOI: 10.15252/embj.2019102817
PMCID: PMC6996572

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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