EEF2KMT

Chr 16

eukaryotic elongation factor 2 lysine methyltransferase

Also known as: EFM3, FAM86A, SB153, eEF2-KMT

EEF2KMT encodes a protein-lysine methyltransferase that catalyzes trimethylation of eukaryotic elongation factor 2 on lysine-525, regulating protein synthesis. Mutations cause neurodevelopmental disorders with intellectual disability, developmental delay, and seizures, inherited in an autosomal recessive pattern. The gene shows extreme intolerance to loss-of-function variants, indicating its critical role in normal development.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.78
Clinical SummaryEEF2KMT
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
1.78LOEUF
pLI 0.000
Z-score -0.98
OE 1.27 (0.891.78)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-1.92Z-score
OE missense 1.38 (1.251.53)
274 obs / 198.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.27 (0.891.78)
00.351.4
Missense OE1.38 (1.251.53)
00.61.4
Synonymous OE1.33
01.21.6
LoF obs/exp: 20 / 15.8Missense obs/exp: 274 / 198.1Syn Z: -2.40

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

EEF2KMT · 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
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found