METTL4

Chr 18

methyltransferase 4, N6-adenosine

Also known as: HsT661

METTL4 encodes a methyltransferase that adds methyl groups to adenine residues in both RNA and DNA, regulating RNA splicing through snRNA modification and controlling mitochondrial transcript levels and DNA copy number. Mutations cause autosomal recessive intellectual disability with microcephaly and seizures, typically presenting in early childhood. This gene is not highly constrained against loss-of-function variants.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
21
Pubs (1 yr)
133
P/LP submissions
0%
P/LP missense
0.95
LOEUF
Mechanism
Clinical SummaryMETTL4
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
127 unique Pathogenic / Likely Pathogenic· 76 VUS of 221 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.95LOEUF
pLI 0.000
Z-score 1.73
OE 0.62 (0.410.95)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.39Z-score
OE missense 0.93 (0.831.04)
223 obs / 240.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.62 (0.410.95)
00.351.4
Missense OE0.93 (0.831.04)
00.61.4
Synonymous OE1.01
01.21.6
LoF obs/exp: 15 / 24.2Missense obs/exp: 223 / 240.1Syn Z: -0.07

ClinVar Variant Classifications

221 submitted variants in ClinVar

Classification Summary

Pathogenic124
Likely Pathogenic3
VUS76
Likely Benign1
124
Pathogenic
3
Likely Pathogenic
76
VUS
1
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
124
0
124
Likely Pathogenic
1
0
2
0
3
VUS
0
54
22
0
76
Likely Benign
0
0
1
0
1
Benign
0
0
0
0
0
Total1541490204

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

METTL4 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →