Article
Unravelling the Effects of the Mutation m.3571insC/MT-ND1 on Respiratory Complexes Structural Organization.
International journal of molecular sciences - 7 Mar 2018
Iommarini Luisa, Ghelli Anna, Tropeano Concetta Valentina, Kurelac Ivana, Leone Giulia, Vidoni Sara, Lombes Anne, Zeviani Massimo, Gasparre Giuseppe, Porcelli Anna Maria
Abstract excerpt
Mammalian respiratory complex I (CI) biogenesis requires both nuclear and mitochondria-encoded proteins and is mostly organized in respiratory supercomplexes. Among the CI proteins encoded by the mitochondrial DNA, NADH-ubiquinone oxidoreductase chain 1 (ND1) is a core subunit, evolutionary conserved from bacteria to mammals. Recently, ND1 has been recognized as a pivotal subunit in maintaining the structural and...
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