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Figure 4 Mitochondrial respiration is affected in lymphoblastoid cell lines (LCLs) of ACO2 mutation carriers Mitochondrial respiration is affected in lymphoblastoid.

Published byPekka Auvinen Modified over 6 years ago

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Presentation on theme: "Figure 4 Mitochondrial respiration is affected in lymphoblastoid cell lines (LCLs) of ACO2 mutation carriers Mitochondrial respiration is affected in lymphoblastoid."— Presentation transcript:

1 Figure 4 Mitochondrial respiration is affected in lymphoblastoid cell lines (LCLs) of ACO2 mutation carriers Mitochondrial respiration is affected in lymphoblastoid cell lines (LCLs) of ACO2 mutation carriers Mitochondrial respiration was measured in isolated mitochondria from homozygous patient carriers (ACO2F414V/F414V), heterozygous ACO2 mutation carrier (ACO2F414V/c), and healthy control cell lines (ACO2c/c) by high-resolution respirometry. (A) State 3 (complex I–linked ADP-coupled respiration) with pyruvate and malate as substrates and (B) State 4u (maximum uncoupled respiration induced by FCCP after ATP synthase inhibition by oligomycin) are depicted. Respiratory efficiency is shown as (C) the respiratory control ratio of ADP-activated flux measuring coupled OXPHOS capacity (state 3/state 4) and (D) the efficiency of FCCP (maximum uncoupled respiration efficiency) (state 4u/state 4). Data are expressed as mean ± SD, n = 3–6 independent experiments per condition, with in total 6–11 measurements per condition; Tukey post hoc results for the one-way analysis of variance are indicated. *p < 0.05, **p < 0.01. Christian G. Bouwkamp et al. Neurol Genet 2018;4:e223 Copyright © 2018 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology

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