EEF1A2

Chr 20

eukaryotic translation elongation factor 1 alpha 2

Also known as: DEE33, EEF1AL, EF-1-alpha-2, EF1A, EIEE33, HS1, MRD38, STN

The protein catalyzes the enzymatic delivery of aminoacyl tRNAs to the ribosome as part of the elongation factor-1 complex and is specifically expressed in brain, heart, and skeletal muscle. Loss-of-function mutations cause developmental and epileptic encephalopathy 33 and autosomal dominant intellectual developmental disorder 38 through an autosomal dominant inheritance pattern. The high intolerance to loss-of-function variants (pLI 0.996, LOEUF 0.186) reflects the protein's essential role in translation elongation during protein synthesis.

ResearchSummary from RefSeq, OMIM, UniProt, Mechanism
GOFmechanismLOEUF 0.19
Clinical SummaryEEF1A2
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Gene-Disease Validity (ClinGen)
complex neurodevelopmental disorder · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
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ClinVar Variants
67 unique Pathogenic / Likely Pathogenic· 172 VUS of 452 total submissions
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Clinical Trials
12 active or recruiting trials — potential therapeutic options may be available
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.19LOEUF
pLI 0.996
Z-score 3.71
OE 0.00 (0.000.19)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
4.82Z-score
OE missense 0.23 (0.180.28)
69 obs / 306.2 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.00 (0.000.19)
00.351.4
Missense OE0.23 (0.180.28)
00.61.4
Synonymous OE0.91
01.21.6
LoF obs/exp: 0 / 16.0Missense obs/exp: 69 / 306.2Syn Z: 0.83
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongEEF1A2-related infantile epileptic encephalopathyGOFAD
DN
0.4587th %ile
GOF
0.4381th %ile
LOF
0.72top 10%

This gene has evidence for multiple mechanisms of pathogenicity (loss-of-function and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to loss-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

LOFprediction above median · 1 literature citation · LOEUF 0.19
GOF1 literature citation

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.

Literature Evidence

GOFRecapitulation of the EEF1A2 D252H neurodevelopmental disorder-causing missense mutation in mice reveals a toxic gain of function.PMID:32160274
LOFPathogenic variants appear to act via a haploinsufficiency mechanism, disrupting both the protein synthesis and integrated stress response functions of EEF1A2.PMID:32196822

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

452 submitted variants in ClinVar

Classification Summary

Pathogenic38
Likely Pathogenic29
VUS172
Likely Benign177
Benign19
Conflicting11
38
Pathogenic
29
Likely Pathogenic
172
VUS
177
Likely Benign
19
Benign
11
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
5
33
0
38
Likely Pathogenic
0
19
10
0
29
VUS
7
128
34
3
172
Likely Benign
0
3
68
106
177
Benign
0
1
12
6
19
Conflicting
11
Total7156157115446

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

View in ClinVar →

Protein Context — Lollipop Plot

EEF1A2 · 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

Healthy Population

Italian Digital Primary Cardiovascular Prevention Study

ACTIVE NOT RECRUITING
NCT05339841Phase NACentro Cardiologico MonzinoStarted 2022-06-10
Mobile health application
Primary Sclerosing CholangitisInflammatory Bowel Diseases

Statin Therapy in Primary Sclerosing Cholangitis (PSC): a Multi-omics Study

RECRUITING
NCT05912387Phase EARLY_PHASE1Stanford UniversityStarted 2023-05-31
Rosuvastatin
Heart Diseases

MyGeneRank: A Digital Platform for Next-Generation Genetic Studies

RECRUITING
NCT03277365Phase NAScripps Translational Science InstituteStarted 2017-09-26
Receive genetic risk information
Pathologic Stage IIIA Cutaneous Melanoma AJCC v8Pathologic Stage IIA Cutaneous Melanoma AJCC v8Pathologic Stage IIB Cutaneous Melanoma AJCC v8

Atorvastatin for Preventing Disease Metastasis in Patients With Resected High-Risk Stage IIA, IIB, or IIIA Melanoma

ACTIVE NOT RECRUITING
NCT06157099Phase PHASE2OHSU Knight Cancer InstituteStarted 2024-09-01
AtorvastatinPlacebo AdministrationComputed Tomography
Cirrhosis

Multi-Center Study of the Effects of Simvastatin on Hepatic Decompensation and Death in Subjects Presenting With High-Risk Compensated Cirrhosis

ACTIVE NOT RECRUITING
NCT03654053Phase PHASE3VA Office of Research and DevelopmentStarted 2020-10-02
Placebo Oral TabletSimvastatin 40mg
Familial Hypercholesterolemia

Effect of Breastfeeding on Lipid Profile and Cardiovascular Risk Markers in Women With Familial Hypercholesterolemia

ACTIVE NOT RECRUITING
NCT05367310Oslo University HospitalStarted 2022-05-01
PRS-based Primary Prevention of CVD

Polygenic Risk Driven Pragmatic Statin Trial for Heart Disease Prevention

ENROLLING BY INVITATION
NCT06820086Phase PHASE4Mikk JÜRISSONStarted 2025-04
Rosuvastatin 20mg
Intracranial AtherosclerosisCognitive ImpairmentCerebrovascular Event

Cognitive Decline and Underlying Mechanisms in Symptomatic Intracranial Artery Stenosis Patients: A Cohort Study

RECRUITING
NCT06336174Anhui Medical UniversityStarted 2022-11-01
Aspirin Tablet, Clopidogrel Bisulfate Tablets and AtorvastatinEndovascular therapy,Aspirin Tablet, Clopidogrel Bisulfate Tablets and Atorvastatin
Gene PolymorphismASCVD

NPC1L1 Gene Polymorphism and the Efficacy and Safety of Hybutimibe

NOT YET RECRUITING
NCT06641661Qianfoshan HospitalStarted 2024-11-01
Hybutimibe 10mg QD
Kidney Neoplasms

MRI Functional Imaging Characteristics and Fat Quantification of CT-fat-free Renal Neoplasms: Relationships With Histological Classifications and Molecular Markers

ACTIVE NOT RECRUITING
NCT06126159Phase NAChang Gung Memorial HospitalStarted 2019-02-11
multiparametric and fat-detection magnetic resonance imaging (MRI)
Preeclampsia (PE)Intrauterine Growth Restriction (IUGR)Placental Insufficiency

Statin Intervention for Severe Early-Onset Placental Insufficiency. (STATIN-PRE Trial)

RECRUITING
NCT07098975Phase PHASE2Fundació Institut de Recerca de l'Hospital de la Santa Creu i Sant PauStarted 2026-01-14
Pravastatin 40 mgPlacebo
Type 1 Diabetes

Cholesterol Lowering and Residual Risk in Diabetes, Type 1

RECRUITING
NCT05641753Phase PHASE4NYU Langone HealthStarted 2022-12-06
Evolocumab CartridgeAtorvastatin Calcium TabletsEzetimibe Tablets
Clinical Literature
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