Modomics - A Database of RNA Modifications

Full name: tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial
Synonym: NOL1/NOP2/Sun domain family member 3
GI: None
Orf:
COG:
UniProt:
Structures: | |
Complex:
Enzyme type: methyltransferase
Position of modification - modification:

Comments:



Protein sequence:

MLTQLKAKSEGKLAKQICKVVLDHFEKQYSKELGDAWNTVREILTSPSCWQYAVLLNRFNYPFELEKDLHLKGYHTLSQGSLPNYPKSVKCYLSRTPGRI
PSERHQIGNLKKYYLLNAASLLPVLALELRDGEKVLDLCAAPGGKSIALLQCACPGYLHCNEYDSLRLRWLRQTLESFIPQPLINVIKVSELDGRKMGDA
QPEMFDKVLVDAPCSNDRSWLFSSDSQKASCRISQRRNLPLLQIELLRSAIKALRPGGILVYSTCTLSKAENQDVISEILNSHGNIMPMDIKGIARTCSH
DFTFAPTGQECGLLVIPDKGKAWGPMYVAKLKKSWSTGKW


Diseases connected to this enzyme:

Description Reaction Disease Name
NSUN3-dependent modification of mt-tRNAMet is a necessary intermediate step towards the formation of 5-formylcytosine (f5C) in mtRNA C:m5C
Combined mitochondrial respiratory chain complex deficiency

Publications:

Title Authors Journal Details PubMed Id DOI
NSUN3 methylase initiates 5-formylcytidine biogenesis in human mitochondrial tRNA(Met). Nakano S Nat Chem Biol. [details] 27214402 10.1038/nchembio.2099
Deficient methylation and formylation of mt-tRNA(Met) wobble cytosine in a patient carrying mutations in NSUN3. Van Haute L, Dietmann S, Kremer L, Hussain S, Pearce SF, Powell CA, Rorbach J, Lantaff R, Blanco S, Sauer S, Kotzaeridou U, Hoffmann GF, Memari Y, Kolb-Kokocinski A, Durbin R, Mayr JA, Frye M, Prokisch H, Minczuk M Nat Commun. [details] 27356879 10.1038/ncomms12039
NSUN3 and ABH1 modify the wobble position of mt-tRNAMet to expand codon recognition in mitochondrial translation. Haag S, Sloan KE, Ranjan N, Warda AS, Kretschmer J, Blessing C, Hübner B, Seikowski J, Dennerlein S, Rehling P, Rodnina MV, Höbartner C, Bohnsack MT ENBO J. [details] 27497299 10.15252/embj.201694885

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