METTL14

Chr 4

methyltransferase 14, N6-adenosine-methyltransferase non-catalytic subunit

Also known as: hMETTL14

METTL14 forms a heterodimer with METTL3 that functions as an N6-methyltransferase complex, methylating adenosine residues in mRNAs to regulate mRNA stability, circadian clock function, and cortical neurogenesis. Biallelic mutations cause developmental delay, intellectual disability, and microcephaly with autosomal recessive inheritance. The gene is highly constrained against loss-of-function variants (LOEUF 0.607), reflecting its essential role in neurodevelopment and cellular differentiation.

Summary from RefSeq, UniProt
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1
Active trials
306
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.61
LOEUF
DN
Mechanism· predicted
Clinical SummaryMETTL14
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.61LOEUF
pLI 0.001
Z-score 3.15
OE 0.36 (0.220.61)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
2.74Z-score
OE missense 0.51 (0.440.59)
124 obs / 244.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.36 (0.220.61)
00.351.4
Missense OE0.51 (0.440.59)
00.61.4
Synonymous OE0.95
01.21.6
LoF obs/exp: 10 / 27.9Missense obs/exp: 124 / 244.5Syn Z: 0.36
DN
0.6357th %ile
GOF
0.4086th %ile
LOF
0.4726th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

METTL14 · protein map & ClinVar variants

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

Clinical Literature
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