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. 2022 Dec 7;12(12):220274. doi: 10.1098/rsob.220274

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© 2022 The Authors.

Published by the Royal Society under the terms of the Creative Commons Attribution License http://creativecommons.org/licenses/by/4.0/, which permits unrestricted use, provided the original author and source are credited.

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Figure 1. — Categorical organization of mitochondrial genes associated with secondary mitochondrial disease (SMD). Genes with deleterious mutations impacting mitochondrial functions secondary to OXPHOS have been broadly classified into three main categories: (1) Molecular pathways related to protein biogenesis, including protein import, protein quality control and Fe-S cluster biogenesis (indicated in blue); (2) Metabolic pathways involving metabolite transport, metabolism of toxic compounds, enzymatic cofactors, TCA cycle metabolism and lipid homeostasis (indicated in green); and (3) Organellar pathways linked to mitochondrial health, including mitochondrial morphology and apoptosis (indicated in red). Genes linked to SMD with currently unclear functions are listed in the Unclear category (indicated in grey). OMM, outer mitochondrial membrane; IMS, intermembrane space; IMM, inner mitochondrial membrane.