Modomics - A Database of RNA Modifications

ID Card:

Full name: tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial
Synonym: NOL1/NOP2/Sun domain family member 3
GI: 74733593
UniProt: Q9H649
Alpha Fold Predicted Structure: AF-Q9H649-F1
Enzyme type: methyltransferase



Protein sequence:

MLTQLKAKSEGKLAKQICKVVLDHFEKQYSKELGDAWNTVREILTSPSCWQYAVLLNRFNYPFELEKDLHLKGYHTLSQGSLPNYPKSVKCYLSRTPGRIPSERHQIGNLKKYYLLNAASLLPVLALELRDGEKVLDLCAAPGGKSIALLQCACPGYLHCNEYDSLRLRWLRQTLESFIPQPLINVIKVSELDGRKMGDAQPEMFDKVLVDAPCSNDRSWLFSSDSQKASCRISQRRNLPLLQIELLRSAIKALRPGGILVYSTCTLSKAENQDVISEILNSHGNIMPMDIKGIARTCSHDFTFAPTGQECGLLVIPDKGKAWGPMYVAKLKKSWSTGKW

Comments:





Alpha Fold Predicted Structure:






Clear Selection and Reset Camera

Protein sequence:

M L T Q L K A K S E G K L A K Q I C K V V L D H F E K Q Y S K E L G D A W N T V R E I L T S P S C W Q Y A V L L N R F N Y P F E L E K D L H L K G Y H T L S Q G S L P N Y P K S V K C Y L S R T P G R I P S E R H Q I G N L K K Y Y L L N A A S L L P V L A L E L R D G E K V L D L C A A P G G K S I A L L Q C A C P G Y L H C N E Y D S L R L R W L R Q T L E S F I P Q P L I N V I K V S E L D G R K M G D A Q P E M F D K V L V D A P C S N D R S W L F S S D S Q K A S C R I S Q R R N L P L L Q I E L L R S A I K A L R P G G I L V Y S T C T L S K A E N Q D V I S E I L N S H G N I M P M D I K G I A R T C S H D F T F A P T G Q E C G L L V I P D K G K A W G P M Y V A K L K K S W S T G K W

Secondary Structure Alphabet

  • G: 3-turn helix (310helix)
  • H: α-helix
  • I: 𝝅-helix (5 - turn helix)
  • T: Hydrogen Bonded Turn
  • B: β-sheet
  • S: Bend
  • C: Coil (residues not present in any of the above conformations)
  • N: Not assigned

Download PDB Structures & DSSP Secondary Structures:

Alpha Fold Pdb Files   AF-Q9H649-F1.pdb  
Alpha Fold Pdbx/mmCIF Files   AF-Q9H649-F1.cif  
DSSP Secondary Structures   Q9H649.dssp  





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