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. 2016 Apr 25;3(6):408–421. doi: 10.1002/acn3.305

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© 2016 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association.

This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial‐NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

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Redox state is imbalanced in a dominant optic atrophy (DOA) mice model. (A) Histograms representative of OPA1 protein quantities, assessed by immunoblot, in cortices of 4 and 10 months old control mice (gray bars) and Opa1 ^+/− mice (striped bars). OPA1 quantities are significantly decreased in both Opa1 ^+/− 4 (0.70 ± 0.10 arbitrary units [AU]) and 10 (0.50 ± 0.05 AU) months old mice when compare to 4 (1.09 ± 0.10 AU) and 10 (1.01 ± 0.09 AU) months old Opa1 ^+/+ mice. Results are expressed as mean ± standard error of the mean (SEM) for 4 and 10 months old mice (n = 6–9). Statistical significance was determined by a two‐way analysis of variance (ANOVA), *P < 0.05, **P < 0.01. (B) Histogram representative of aconitase activities in cortices of 4 and 10 months old Opa1 ^+/− (striped bars) and Opa1 ^+/+ control mice (gray bars) cortices. Aconitase activity is significantly lower in 4 (1.56 ± 0.11 mU/mg) and 10 (0.97 ± 0.23 mU/mg) months old Opa1 ^+/− mice when compared to 4 (2.95 ± 0.40 mU/mg) and 10 (2.24 ± 0.38 mU/mg) months old control Opa1 ^+/+ mice, respectively. Aconitase proteins quantities are unchanged in DOA mice model's cortices. Results are expressed as mean ± SEM (n = 9 for 4 months old mice and n = 6 for 10 months old mice). Statistical significance was determined by a two‐way ANOVA, *P < 0.05. (C) Histogram representative of SOD1, SOD2, and catalase proteins quantities relative to actin, assessed by immunoblot, in 4 and 10 months old Opa1 ^+/− (striped bars) and Opa1 ^+/+ (gray bars) mice cortices. SOD1 proteins quantities are significantly increased in 10 months old (1.48 ± 0.17 AU) Opa1 ^+/− mice compared to 4 months old (0.76 ± 0.08 AU) Opa1 ^+/− mice. Likewise, SOD2 proteins quantities are significantly increased in 10 months old (1.55 ± 0.11 AU) Opa1 ^+/− mice compared to 4 months old (0.98 ± 0.09 AU) Opa1 ^+/− mice. Catalase quantity is unchanged in Opa1 ^+/+ and Opa1 ^+/− 4 and 10 months old mice. Results are expressed as mean ± SEM (n = 9 for 4 months old mice and n = 6 for 10 months old mice). Statistical significance was determined using a two‐way ANOVA, *P < 0.05, **P < 0.01.