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New Insights on Rotenone Resistance of Complex I Induced by the m.11778G>A/MT-ND4 Mutation Associated with Leber's Hereditary Optic Neuropathy.

Molecules (Basel, Switzerland) - 16 Feb 2022

Musiani Francesco, Rigobello Laura, Iommarini Luisa, Carelli Valerio, Degli Esposti Mauro, Ghelli Anna Maria

Abstract excerpt

The finding that the most common mitochondrial DNA mutation m.11778G>A/MT-ND4 (p.R340H) associated with Leber's hereditary optic neuropathy (LHON) induces rotenone resistance has produced a long-standing debate, because it contrasts structural evidence showing that the ND4 subunit is far away from the quinone-reaction site in complex I, where rotenone acts. However, recent cryo-electron microscopy data revealed...

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Identifiers

PMID: 35209128
DOI: 10.3390/molecules27041341
PMCID: PMC8876992

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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