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. 2023 Nov 14;9(6):e200106. doi: 10.1212/NXG.0000000000200106

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Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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(A) Immunofluorescence staining shows fragmentation of mitochondria in the patient fibroblasts. The α-MPP levels are lower in the patient, but analysis of fluorescence profiles (white line in the merged images) shows the correct localization of α-MPP (green) in mitochondria (red). (B) Mitochondrial morphometry measurements confirm statistically significant differences in the mitochondrial morphology of the patient and control fibroblasts. Each dot represents the average value for a single measured cell. α-MPP = α-subunit of mitochondrial processing peptidase.