tRNA methyltransferase 10A (TRMT10A)

tRNA methyltransferase 10A (TRMT10A) is an S-adenosylmethionine-dependent enzyme that catalyzes the N¹-methylation of guanine at position 9 (m¹G9) in a subset of nuclear-encoded cytosolic tRNAs[1][2][4][6]. This posttranscriptional modification is essential for tRNA stability and function, thereby safeguarding accuracy in protein synthesis. TRMT10A is ubiquitously expressed, with particular enrichment in the developing brain and pancreatic islets. Loss-of-function mutations in TRMT10A cause a syndromic disorder characterized by microcephaly, intellectual disability, short stature, and early-onset diabetes, likely due to impaired tRNA modification leading to translation defects, oxidative stress, and cell death in β-cells and neurons[1][4][6]. TRMT10A's activity is also functionally coordinated with mRNA modification pathways, notably via interaction with the mRNA demethylase FTO, indicating broader roles in the regulation of gene expression[5]. **Note:** There are currently no known drugs that directly target TRMT10A, nor are there established biomarkers or drug mechanisms of action related to this enzyme[6]. All names listed are valid aliases; the most commonly accepted abbreviation is TRMT10A. The major molecular classification is as an enzyme (tRNA methyltransferase), not as a traditional "receptor."

Other names

TRMT10ARG9MTD2MGC27034TRM10RNA (guanine-9-)-methyltransferase domain-containing protein 2HEL-S-88MSSGMMSSGM1tRNA (guanine(9)-N(1))-methyltransferase TRMT10Aepididymis secretory protein Li 88