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. 2015 Mar 13;2(5):492–509. doi: 10.1002/acn3.189

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© 2015 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 License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

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Analysis of ECHS1 levels and enzymatic activity. (A) Analysis of ECHS1 steady state levels by immunoblotting show a decrease in the amount of ECHS1 in patient-derived cell lines compared to controls. (B) Analysis of residual ECHS1 enzymatic activity indicates reduced 2-enoyl-CoA hydratase activity in cell lysates from patients’ fibroblasts compared to controls. Results shown are from at least three experiments performed in triplicates and controls (n = 4). Boxplot whiskers indicate range from 5th to 95th percentile. *P < 0.001; two-tailed unpaired t-test. (C) Analysis of palmitate-dependent OCR in fibroblast cell lines revealed impaired respiration in patients’ cells in comparison to controls. The experiment was performed several times with very similar results. The data are shown from one experiment performed with more than 10 replicates for each cell line grown and treated in parallel. Error bars indicate 1 SD. *P < 0.001; two-tailed unpaired t-test. ECHS1, short-chain enoyl-CoA hydratase; OCR, oxygen consumption rate.