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dc.contributor.authorVan Haute, Lindseyen
dc.contributor.authorPowell, Chrisen
dc.contributor.authorMinczuk, Michalen
dc.date.accessioned2017-05-11T15:31:19Z
dc.date.available2017-05-11T15:31:19Z
dc.date.issued2017-03-02en
dc.identifier.issn2218-273X
dc.identifier.urihttps://www.repository.cam.ac.uk/handle/1810/264195
dc.description.abstractHuman mitochondria contain their own genome, which uses an unconventional genetic code. In addition to the standard AUG methionine codon, the single mitochondrial tRNA Methionine (mt‐tRNA$^{Met}$) also recognises AUA during translation initiation and elongation. Post‐transcriptional modifications of tRNAs are important for structure, stability, correct folding and aminoacylation as well as decoding. The unique 5‐formylcytosine (f$^{5}$C) modification of position 34 in mt‐tRNA$^{Met}$ has been long postulated to be crucial for decoding of unconventional methionine codons and efficient mitochondrial translation. However, the enzymes responsible for the formation of mitochondrial f$^{5}$C have been identified only recently. The first step of the f$^{5}$C pathway consists of methylation of cytosine by NSUN3. This is followed by further oxidation by ABH1. Here, we review the role of f$^{5}$C, the latest breakthroughs in our understanding of the biogenesis of this unique mitochondrial tRNA modification and its involvement in human disease.
dc.description.sponsorshipMedical Research Council, UK is gratefully acknowledged for generous support of this work.
dc.languageengen
dc.language.isoenen
dc.publisherMDPI
dc.rightsAttribution 4.0 Internationalen
dc.rightsAttribution 4.0 Internationalen
dc.rightsAttribution 4.0 Internationalen
dc.rightsAttribution 4.0 Internationalen
dc.rightsAttribution 4.0 Internationalen
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/en
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/en
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/en
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/en
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/en
dc.subjectmitochondriaen
dc.subjecttRNAen
dc.subjectNSUN3en
dc.subject5‐methylcytosineen
dc.subject5‐formylcytosineen
dc.subjectRNA modificationen
dc.subjecttranslationen
dc.titleDealing with an Unconventional Genetic Code in Mitochondria: The Biogenesis and Pathogenic Defects of the 5‐Formylcytosine Modification in Mitochondrial tRNA$^{Met}$en
dc.typeArticle
prism.issueIdentifier1en
prism.number24en
prism.publicationDate2017en
prism.publicationNameBiomoleculesen
prism.volume7en
dc.identifier.doi10.17863/CAM.9554
dcterms.dateAccepted2017-02-24en
rioxxterms.versionofrecord10.3390/biom7010024en
rioxxterms.versionVoRen
rioxxterms.licenseref.urihttp://creativecommons.org/licenses/by/4.0/en
rioxxterms.licenseref.startdate2017-03-02en
dc.contributor.orcidMinczuk, Michal [0000-0001-8242-1420]
dc.identifier.eissn2218-273X
rioxxterms.typeJournal Article/Reviewen
pubs.funder-project-idMRC (MC_U105697135)
pubs.funder-project-idMRC (MC_UU_00015/4)


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Attribution 4.0 International
Except where otherwise noted, this item's licence is described as Attribution 4.0 International