Full name: | " tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial" |
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UniProt: | Q8CCT7 |
Alpha Fold Predicted Structure: | AF-Q8CCT7-F1 |
Enzyme type: | methyltransferase |
MLTRLKAKSEGKLAKQLCRVVLDQFDKQYSKELGDSWSTVRDVLISPSLWQYAILFNRFNYPFELEKALHLRGYHTVLQGALPHYPKSMKCYLSRTPDRMPSERHQTGSLKKYYLLNAASLLPVLALELRDGEAVLDLCAAPGGKSVALLQCAYPGYLLCNEYDRPRGRWLRQTLESFIPQPLINVIKVSELDGREMGDAQPATFDKVLVDAPCSNDRSWLFSSDSQKAAYRIHQRKNLPVLQVELVRSAIKALRPGGLLVYSTCTLSKAENQDVISEVLTSDSNIVPVDISGIARTFSQDFTFAPTDQKCSLLVIPEKGKAWGPMYIAKLKKGMSTRKRQGEFCKPC
A mitochondrial tRNA modification enzyme, NSUN3, is found to be essential for embryonic development in mice, with whole-body knockout causing embryonic lethality and heart-specific Nsun3 knockout leading to heart aberration in older mice. Human NSUN3 mutations are associated with mitochondrial diseases. Mitochondrial tRNA methyltransferase that mediates methylation of cytosine to 5-methylcytosine (m5C) at position 34 of mt-tRNA(Met). mt-tRNA(Met) methylation at cytosine34 takes place at the wobble position of the anticodon and initiates the formation of 5-formylcytosine (f5c) at this position. mt-tRNA(Met) containing the f5c modification at the wobble position enables recognition of the AUA codon in addition to the AUG codon, expanding codon recognition in mitochondrial translation.
Alpha Fold Pdb Files | AF-Q8CCT7-F1.pdb   |
Alpha Fold Pdbx/mmCIF Files | AF-Q8CCT7-F1.cif   |
DSSP Secondary Structures | Q8CCT7.dssp   |
Title | Authors | Journal | Details | ||
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NSUN3-mediated mitochondrial tRNA 5-formylcytidine modification is essential for embryonic development and respiratory complexes in mice | Yoshitaka Murakami 1 2, Fan-Yan Wei 3, Yoshimi Kawamura 4, Haruki Horiguchi 5, Tsuyoshi Kadomatsu 5, Keishi Miyata 5, Kyoko Miura 4 6, Yuichi Oike 5 6, Yukio Ando 7, Mitsuharu Ueda 2 6, Kazuhito Tomizawa 8 9, Takeshi Chujo | [details] | 36949224 | 10.1038/s42003-023-04680-x |
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