Screening strategy using a filamentous fungus model to repurpose drugs for mitochondrial complex I deficiencies.

doi:10.1016/j.lfs.2026.124251

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Article

Screening strategy using a filamentous fungus model to repurpose drugs for mitochondrial complex I deficiencies.

Life sciences - 1 Apr 2026

Sellem Carole H, Bounaix Nolwenn, Logerais Mathilde, Renaud Aurélie, d'Elia Marc Alexandre, Richard Jeremy, Almyre Claire, Becker Guillaume, Rivron Jordan, Hoarau Anaïs, Gueguen Naïg, Desquiret-Dumas Valérie, Inisan Aurore, Belal Sophie, Mellinger Adélie, Godard François, Paquis-Flucklinger Véronique, Baris Olivier R, Azoulay Stéphane, Delahodde Agnès, Tribouillard-Tanvier Déborah, Bonnefoy Nathalie, Procaccio Vincent

Abstract excerpt

AIM: This study aimed to repurpose FDA-approved drugs for the treatment of mitochondrial complex I diseases. MATERIALS AND METHODS: The NUO-51 protein of the filamentous fungus Podospora anserina is the homolog of the human key catalytic subunit of complex I, NDUFV1. By introducing a pathogenic mutation into P. anserina NUO-51 we created a novel model of complex I deficiency targeting the NDUFV1 subunit. The...

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PMID: 41644045
DOI: 10.1016/j.lfs.2026.124251

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NDUFV1
DAP

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