TRMT10A

Chr 4AR

tRNA methyltransferase 10A

Also known as: HEL-S-88, MSSGM, MSSGM1, RG9MTD2, TRM10

This protein is an S-adenosyl-L-methionine-dependent methyltransferase that catalyzes the formation of N(1)-methylguanine at position 9 in tRNAs. Biallelic mutations cause autosomal recessive microcephaly, short stature, and impaired glucose metabolism. The gene is highly tolerant to loss-of-function variation in the general population (very low pLI score).

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.181 OMIM phenotype
Clinical SummaryTRMT10A
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.18LOEUF
pLI 0.000
Z-score 0.91
OE 0.79 (0.541.18)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.44Z-score
OE missense 0.91 (0.801.03)
166 obs / 182.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.79 (0.541.18)
00.351.4
Missense OE0.91 (0.801.03)
00.61.4
Synonymous OE0.80
01.21.6
LoF obs/exp: 17 / 21.5Missense obs/exp: 166 / 182.6Syn Z: 1.24

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TRMT10A · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC