Modomics - A Database of RNA Modifications

Full name:	tRNA (adenine(58)-N(1))-methyltransferase, mitochondrial
Synonym:
GI:	222831587
Orf:
COG:	COG2519
UniProt:	Q9BVS5
Structures:	\| 2B25 \|
Complex:
Enzyme type:	methyltransferase
Position of modification - modification:	t:58 - m1A

Comments:

Paralog of cytoplasmic Trm61A. Unlike its cytoplasmic paralog that consists of an alpha2beta2 heterotetramer formed by Trmt61A and Trmt6, Trmt61B forms a homo-oligomer (presumably a homotetramer).

Protein sequence:

MLMAWCRGPVLLCLRQGLGTNSFLHGLGQEPFEGARSLCCRSSPRDLRDGEREHEAAQRKAPGAESCPSLPLSISDIGTGCLSSLENLRLPTLREESSPR
ELEDSSGDQGRCGPTHQGSEDPSMLSQAQSATEVEERHVSPSCSTSRERPFQAGELILAETGEGETKFKKLFRLNNFGLLNSNWGAVPFGKIVGKFPGQI
LRSSFGKQYMLRRPALEDYVVLMKRGTAITFPKDINMILSMMDINPGDTVLEAGSGSGGMSLFLSKAVGSQGRVISFEVRKDHHDLAKKNYKHWRDSWKL
SHVEEWPDNVDFIHKDISGATEDIKSLTFDAVALDMLNPHVTLPVFYPHLKHGGVCAVYVVNITQVIELLDGIRTCELALSCEKISEVIVRDWLVCLAKQ
KNGILAQKVESKINTDVQLDSQEKIGVKGELFQEDDHEESHSDFPYGSFPYVARPVHWQPGHTAFLVKLRKVKPQLN

Enzymatic activities:

Reaction	Substrate	Type	Position
A:m1A	tRNA (t)	Leu/ÊAA/mitochondrion	58
A:m1A	tRNA (t)	Ser/GCU/mitochondrion	58
A:m1A	tRNA (t)	Lys/unknown/eukaryotic cytosol	58

Diseases connected to this enzyme:

Description	Reaction	Disease Name
Missing m1A modification on mitochondrial tRNA at the 58 position is associated with the MERRF disease. m1A58 provides a positive charge on tRNA tertiary structure that affect the binding of elongation factor which delivers tRNA to ribosome.	A:m1A	Myoclonus epilepsy, ragged-red fibers (MERRF)

Publications:

Title	Authors	Journal	Details	PubMed Id	DOI
Trmt61B is a methyltransferase responsible for 1-methyladenosine at position 58 of human mitochondrial tRNAs.	Chujo T, Suzuki T...	RNA	[details]	23097428	-

Links:

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