Modomics - A Database of RNA Modifications

ID Card:

Full name: Dimethyladenosine transferase 1, mitochondrial
Synonym: h-mtTFB, CGI75
GI: 315013580
UniProt: Q8WVM0
Structures: | 6AAX | 6AJK |
Alpha Fold Predicted Structure: AF-Q8WVM0-F1
Enzyme type: methyltransferase
Position of modification - modification: s:936(None) - m6,6A
s:937(None) - m6,6A


PDB Structures:


6AAX

Structure Description:

Title:
Classification:
Technique:

Abstract of the PDB Structure's related Publication:

Mitochondria are essential molecular machinery for the maintenance of cellular energy supply by the oxidative phosphorylation system (OXPHOS). Mitochondrial transcription factor B1 (TFB1M) is a dimethyltransferase that maintains mitochondrial homeostasis by catalyzing dimethylation of two adjacent adenines located in helix45 (h45) of 12S rRNA. This m62A modification is indispensable for the assembly and maturation of human mitochondrial ribosomes. However, both the mechanism of TFB1M catalysis and the precise function of TFB1M in mitochondrial homeostasis are unknown. Here we report the crystal structures of a ternary complex of human (hs) TFB1M-h45-S-adenosyl-methionine and a binary complex hsTFB1M-h45. The structures revealed a distinct mode of hsTFB1M interaction with its rRNA substrate and with the initial enzymatic state involved in m62A modification. The suppression of hsTFB1M protein level or the overexpression of inactive hsTFB1M mutants resulted in decreased ATP production and reduced expression of components of the mitochondrial OXPHOS without affecting transcription of the corresponding genes and their localization to the mitochondria. Therefore, hsTFB1M regulated the translation of mitochondrial genes rather than their transcription via m62A modification in h45.

Download RCSB-PDB Structures:

Pdb Files   6AAX.pdb   6AJK.pdb  
Pdbx/mmCIF Files   6AAX.cif   6AJK.cif  


Protein sequence:

MAASGKLSTCRLPPLPTIREIIKLLRLQAAKQLSQNFLLDLRLTDKIVRKAGNLTNAYVYEVGPGPGGITRSILNADVAELLVVEKDTRFIPGLQMLSDAAPGKLRIVHGDVLTFKVEKAFSESLKRPWEDDPPNVHIIGNLPFSVSTPLIIKWLENISCRDGPFVYGRTQMTLTFQKEVAERLAANTGSKQRSRLSVMAQYLCNVRHIFTIPGQAFVPKPEVDVGVVHFTPLIQPKIEQPFKLVEKVVQNVFQFRRKYCHRGLRMLFPEAQRLESTGRLLELADIDPTLRPRQLSISHFKSLCDVYRKMCDEDPQLFAYNFREELKRRKSKNEEKEEDDAENYRL

Comments:





Alpha Fold Predicted Structure:






Clear Selection and Reset Camera

Protein sequence:

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

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-Q8WVM0-F1.pdb  
Alpha Fold Pdbx/mmCIF Files   AF-Q8WVM0-F1.cif  
DSSP Secondary Structures   Q8WVM0.dssp  





Publications:

Title Authors Journal Details PubMed Id DOI
A human mitochondrial transcription factor is related to RNA adenine methyltransferases and binds S-adenosylmethionine. McCulloch V, Seidel-Rogol BL, Shadel GS. Mol Cell Biol. [details] 11809803 -
Methylation of 12S rRNA is necessary for in vivo stability of the small subunit of the mammalian mitochondrial ribosome. Metodiev MD, Lesko N, Park CB, Cámara Y, Shi Y, Wibom R, Hultenby K, Gustafsson CM, Larsson NG Cell Metab. [details] 19356719 10.1016/j.cmet.2009.03.001