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:

_PubMed_

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