{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T09:02:54Z","timestamp":1783587774494,"version":"3.55.0"},"reference-count":74,"publisher":"Oxford University Press (OUP)","issue":"R1","license":[{"start":{"date-parts":[[2024,1,27]],"date-time":"2024-01-27T00:00:00Z","timestamp":1706313600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Sigrid Juselius Foundation Senior Investigator Award, Research Council of Finland"},{"name":"Hereditary Neuropathy Foundation and Lindsey Flynt, National Ataxia Foundation, and the Magnus Ehrnrooth Foundation"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,5,22]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Human mitochondrial DNA is one of the most simplified cellular genomes and facilitates compartmentalized gene expression. Within the organelle, there is no physical barrier to separate transcription and translation, nor is there evidence that quality control surveillance pathways are active to prevent translation on faulty mRNA transcripts. Mitochondrial ribosomes synthesize 13 hydrophobic proteins that require co-translational insertion into the inner membrane of the organelle. To maintain the integrity of the inner membrane, which is essential for organelle function, requires responsive quality control mechanisms to recognize aberrations in protein synthesis. In this review, we explore how defects in mitochondrial protein synthesis can arise due to the culmination of inherent mistakes that occur throughout the steps of gene expression. In turn, we examine the stepwise series of quality control processes that are needed to eliminate any mistakes that would perturb organelle homeostasis. We aim to provide an integrated view on the quality control mechanisms of mitochondrial protein synthesis and to identify promising avenues for future research.<\/jats:p>","DOI":"10.1093\/hmg\/ddae012","type":"journal-article","created":{"date-parts":[[2024,1,27]],"date-time":"2024-01-27T22:56:35Z","timestamp":1706396195000},"page":"R53-R60","source":"Crossref","is-referenced-by-count":12,"title":["Mitochondrial protein synthesis quality control"],"prefix":"10.1093","volume":"33","author":[{"given":"Lidiia","family":"Koludarova","sequence":"first","affiliation":[{"name":"University of Helsinki Institute of Biotechnology, HiLIFE, , Helsinki 00014, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8136-2753","authenticated-orcid":false,"given":"Brendan J","family":"Battersby","sequence":"additional","affiliation":[{"name":"University of Helsinki Institute of Biotechnology, HiLIFE, , Helsinki 00014, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,5,23]]},"reference":[{"key":"2024052307520069500_ref1","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1038\/nrm.2017.66","article-title":"Mitochondrial diseases: the contribution of organelle stress responses to pathology","volume":"19","author":"Suomalainen","year":"2018","journal-title":"Nat Rev Mol Cell Biol"},{"key":"2024052307520069500_ref2","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1038\/s41582-018-0101-0","article-title":"POLG-related disorders and their neurological manifestations","volume":"15","author":"Rahman","year":"2019","journal-title":"Nat Rev Neurol"},{"key":"2024052307520069500_ref3","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1002\/1873-3468.12962","article-title":"The role of tRNA synthetases in neurological and neuromuscular disorders","volume":"592","author":"Boczonadi","year":"2018","journal-title":"FEBS Lett"},{"key":"2024052307520069500_ref4","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1002\/1873-3468.14049","article-title":"The molecular pathology of pathogenic mitochondrial tRNA variants","volume":"595","author":"Richter","year":"2021","journal-title":"FEBS Lett"},{"key":"2024052307520069500_ref5","article-title":"Mechanisms and regulation of human mitochondrial transcription","author":"Tan","year":"2023","journal-title":"Nat Rev Mol Cell Biol"},{"key":"2024052307520069500_ref6","doi-asserted-by":"crossref","first-page":"1135","DOI":"10.1038\/s41467-021-21279-0","article-title":"POLRMT mutations impair mitochondrial transcription causing neurological disease","volume":"12","author":"Ol\u00e1hov\u00e1","year":"2021","journal-title":"Nat Commun"},{"key":"2024052307520069500_ref7","doi-asserted-by":"crossref","first-page":"1009","DOI":"10.1038\/s41467-023-36277-7","article-title":"TEFM variants impair mitochondrial transcription causing childhood-onset neurological disease","volume":"14","author":"Van Haute","year":"2023","journal-title":"Nat Commun"},{"key":"2024052307520069500_ref8","doi-asserted-by":"crossref","first-page":"18145","DOI":"10.1074\/jbc.M117.797480","article-title":"Transcriptional fidelities of human mitochondrial POLRMT, yeast mitochondrial Rpo41, and phage T7 single-subunit RNA polymerases","volume":"292","author":"Sultana","year":"2017","journal-title":"J Biol Chem"},{"key":"2024052307520069500_ref9","doi-asserted-by":"crossref","first-page":"1547","DOI":"10.1038\/s41467-023-36525-w","article-title":"Evolutionary conservation of the fidelity of transcription","volume":"14","author":"Chung","year":"2023","journal-title":"Nat Commun"},{"key":"2024052307520069500_ref10","doi-asserted-by":"crossref","first-page":"4284","DOI":"10.1093\/nar\/gkq1224","article-title":"TEFM (c17orf42) is necessary for transcription of human mtDNA","volume":"39","author":"Minczuk","year":"2011","journal-title":"Nucleic Acids Res"},{"key":"2024052307520069500_ref11","doi-asserted-by":"crossref","first-page":"2615","DOI":"10.1093\/nar\/gkv105","article-title":"TEFM is a potent stimulator of mitochondrial transcription elongation in vitro","volume":"43","author":"Posse","year":"2015","journal-title":"Nucleic Acids Res"},{"key":"2024052307520069500_ref12","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1126\/science.aaa0986","article-title":"Mitochondrial biology. 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