Trepo - Selaus tekijän mukaan "Gailus-Durner, Valérie"

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    • AOX delays the onset of the lethal phenotype in a mouse model of Uqcrh (complex III) disease 

      Jacobs, Howard T.; Szibor, Marten; Rathkolb, Birgit; da Silva-Buttkus, Patricia; Aguilar-Pimentel, Juan Antonio; Amarie, Oana V.; Becker, Lore; Calzada-Wack, Julia; Dragano, Nathalia; Garrett, Lillian; Gerlini, Raffaele; Hölter, Sabine M.; Klein-Rodewald, Tanja; Kraiger, Markus; Leuchtenberger, Stefanie; Marschall, Susan; Östereicher, Manuela A.; Pfannes, Kristina; Sanz-Moreno, Adrián; Seisenberger, Claudia; Spielmann, Nadine; Stoeger, Claudia; Wurst, Wolfgang; Fuchs, Helmut; Hrabě de Angelis, Martin; Gailus-Durner, Valérie (10 / 2023)
      article
      The alternative oxidase, AOX, provides a by-pass of the cytochrome segment of the mitochondrial respiratory chain when the chain is unavailable. AOX is absent from mammals, but AOX from Ciona intestinalis is benign when ...

    • Broad AOX expression in a genetically tractable mouse model does not disturb normal physiology 

      Szibor, Marten; Dhandapani, Praveen; Dufour, Eric; Holmström, Kira; Zhuang, Yuan; Salwig, Isabelle; Wittig, Ilka; Heidler, Juliana; Gizatullina, Zemfira; Gainutdinov, Timur; German Mouse Clinic Consortium; Fuchs, Helmut; Gailus-Durner, Valérie; Hrabě de Angelis, Martin; Nandania, Jatin; Velagapudi, Vidya; Wietelmann, Astrid; Rustin, Pierre; Gellerich, Frank; Jacobs, Howard T; Braun, Thomas (2017)
      article

    • Knockout of the Complex III subunit Uqcrh causes bioenergetic impairment and cardiac contractile dysfunction 

      Spielmann, Nadine; Schenkl, Christina; Komlódi, Tímea; da Silva-Buttkus, Patricia; Heyne, Estelle; Rohde, Jana; Amarie, Oana V.; Rathkolb, Birgit; Gnaiger, Erich; Doenst, Torsten; Fuchs, Helmut; Gailus-Durner, Valérie; de Angelis, Martin Hrabě; Szibor, Marten (2023)
      article
      Ubiquinol cytochrome c reductase hinge protein (UQCRH) is required for the electron transfer between cytochrome c1 and c of the mitochondrial cytochrome bc1 Complex (CIII). A two-exon deletion in the human UQCRH gene has ...