ELF1

Chr 13

E74 like ETS transcription factor 1

Also known as: EFTUD1, RIA1

ELF1 encodes a transcription factor that regulates gene expression in lymphoid cells and activates specific promoters including LYN and BLK. Mutations cause autosomal dominant immunodeficiency with developmental delay and hypotonia, affecting both immune function and neurological development. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.58).

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.58
Clinical SummaryELF1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.32) despite low pLI — interpret in context.
📋
ClinVar Variants
46 unique Pathogenic / Likely Pathogenic· 87 VUS of 148 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.58LOEUF
pLI 0.011
Z-score 3.12
OE 0.32 (0.190.58)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.91Z-score
OE missense 0.86 (0.780.95)
279 obs / 325.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.32 (0.190.58)
00.351.4
Missense OE0.86 (0.780.95)
00.61.4
Synonymous OE0.95
01.21.6
LoF obs/exp: 8 / 24.7Missense obs/exp: 279 / 325.4Syn Z: 0.47

ClinVar Variant Classifications

148 submitted variants in ClinVar

Classification Summary

Pathogenic46
VUS87
Likely Benign1
Benign2
46
Pathogenic
87
VUS
1
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
46
0
46
Likely Pathogenic
0
0
0
0
0
VUS
0
80
7
0
87
Likely Benign
0
1
0
0
1
Benign
0
0
0
2
2
Total081532136

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

View in ClinVar →

Protein Context — Lollipop Plot

ELF1 · 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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