Article

Respiratory complex III <sub>2</sub> assembles complex I via toxic intermediate in mitochondrial disease

2025-06-18

Abstract excerpt

Mutations in mitochondrial complex I can cause severe metabolic disease. Although no treatments are available for complex I deficiencies, chronic hypoxia improves lifespan and function in a mouse model of the severe mitochondrial disease Leigh syndrome caused by mutation of complex I subunit NDUFS4. To understand the molecular mechanism of NDUFS4 mutant pathophysiology and hypoxia rescue, we investigated the struc...

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Identifiers and source

Literature Corpus work: 959469ae-891c-5ffd-9d9e-89581bceb375
DOI: 10.1101/2025.06.17.660237

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