In mitochondrial disease respiratory complex III2 assembles complex I via toxic intermediate
Maria G. Ayala-Hernandez, Anetzy Bermudez Torales, Hannah Camille Tan, Claire B. Montgomery, Abhilash Padavannil, Gino A. Cortopassi, James A. Letts
The paper identifies toxic intermediate complexes formed during mitochondrial complex I assembly in NDUFS4 mutant mice, revealing hypoxia's role in rescuing disease by preventing pathological reverse electron transfer.
bioRxiv · Jun 17, 2025 · PreprintBiochemistry