Entrez Summary: This gene encodes 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 this gene have been implicated in autosomal recessive intellectual disorder (ARID). Alternative splicing results in multiple transcript variants encoding different isoforms. There is a pseudogene of this gene on the X chromosome. [provided by RefSeq, May 2017].
UniProt Summary: Dimethylates a single guanine residue at position 26 of most tRNAs using S-adenosyl-L-methionine as donor of the methyl groups.
Pfam DomainsGO Terms
Pfam Domains
Met 10
TRM
zf-CCCH
GO Terms
tRNA N2-guanine methylation
tRNA (guanine-N2-)-methyltransferase activity
tRNA methylation
tRNA binding
RNA methylation
tRNA modification
tRNA processing
RNA modification
tRNA metabolic process
macromolecule methylation
methylation
ncRNA processing
ncRNA metabolic process
RNA processing
RNA binding
RNA metabolic process
gene expression
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)