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. 2024 May 15;11(6):1615–1629. doi: 10.1002/acn3.52079

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© 2024 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.

This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

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Respiration profile of COASY mutated fibroblasts. (A) OCR of fibroblasts, expressed as pmoles O₂/min/1000 cells, under basal conditions and after injection of oligomycin (Oligo), carbonyl cyanide 4‐(trifluoromethoxy) phenylhydrazone (FCCP), rotenone (Rot) and antimycin A (Ant). Data are shown as mean ± SD of three control lines (Ctr, n = 22) and patients' cells (Pt, n = 12). (B–D) Maximal (B), basal (C), and ATP‐linked (D) respiration were calculated from OCR traces and are reported in the graphs as mean ± SD. *p < 0.05; **p < 0.01; ***p < 0.001 (one‐way ANOVA).