TRMT1

TRMT1 is a tRNA-modifying enzyme that acts as a dimethyltransferase, modifying a single guanine residue at position 26 of the tRNA. The encoded enzyme has both mono- and dimethylase activity when exogenously expressed, and uses S-adenosyl methionine as a methyl donor. The C-terminal region of the encoded protein has both a zinc finger motif, and an arginine/proline-rich region. Mutations in TRMT1 have been implicated in autosomal recessive intellectual disorder (ARID).

TRNA Methyltransferase 1

Dimethylates a single guanine residue at position 26 of most tRNAs using S-adenosyl-L-methionine as donor of the methyl groups.

Biological Process tRNA modification Source:Reactome
Biological Process tRNA N2-guanine methylation Source:GO_Central1 Publication

nucleoplasm
nucleus

Intellectual developmental disorder, autosomal recessive 68 (MRT68):
A form of intellectual disability, a disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptive behavior and manifested during the developmental period.