CELF1

Chr 11

CUGBP Elav-like family member 1

Also known as: BRUNOL2, CUG-BP, CUGBP, CUGBP1, EDEN-BP, NAB50, NAPOR, hNab50

CELF1 encodes an RNA-binding protein that regulates alternative splicing, mRNA translation, and stability across multiple tissues including heart and muscle. Mutations cause autosomal dominant myotonic dystrophy type 1, a multisystem disorder affecting skeletal muscle, cardiac conduction, and other organ systems with variable age of onset. The gene is highly constrained against loss-of-function variants, reflecting its essential role in RNA processing.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.17
Clinical SummaryCELF1
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.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.17LOEUF
pLI 1.000
Z-score 4.71
OE 0.04 (0.010.17)
Highly constrained

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

Missense Constraint
3.34Z-score
OE missense 0.45 (0.390.52)
128 obs / 287.5 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.04 (0.010.17)
00.351.4
Missense OE0.45 (0.390.52)
00.61.4
Synonymous OE0.86
01.21.6
LoF obs/exp: 1 / 27.8Missense obs/exp: 128 / 287.5Syn Z: 1.14
DN
0.3594th %ile
GOF
0.3789th %ile
LOF
0.84top 5%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.17

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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CELF1 · 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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