CEBPB

Chr 20

CCAAT enhancer binding protein beta

Also known as: C/EBP-beta, IL6DBP, NF-IL6, TCF5

CEBPB encodes a transcription factor with a basic leucine zipper domain that regulates genes involved in immune and inflammatory responses, adipogenesis, gluconeogenesis, and hematopoiesis. Mutations cause autosomal recessive intellectual developmental disorder with dysmorphic facies, seizures, and behavioral abnormalities. The gene shows moderate tolerance to loss-of-function variants (pLI 0.36, LOEUF 1.03).

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
165
Pubs (1 yr)
10
P/LP submissions
0%
P/LP missense
1.02
LOEUF
LOF
Mechanism· predicted
Clinical SummaryCEBPB
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.22) despite low pLI — interpret in context.
📋
ClinVar Variants
10 unique Pathogenic / Likely Pathogenic· 49 VUS of 62 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.02LOEUF
pLI 0.358
Z-score 1.56
OE 0.22 (0.081.02)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.90Z-score
OE missense 0.77 (0.650.92)
92 obs / 119.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.22 (0.081.02)
00.351.4
Missense OE0.77 (0.650.92)
00.61.4
Synonymous OE1.30
01.21.6
LoF obs/exp: 1 / 4.6Missense obs/exp: 92 / 119.6Syn Z: -1.76
DN
0.5575th %ile
GOF
0.3788th %ile
LOF
0.71top 10%

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

LOFprediction above median

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

62 submitted variants in ClinVar

Classification Summary

Pathogenic7
Likely Pathogenic3
VUS49
Likely Benign3
7
Pathogenic
3
Likely Pathogenic
49
VUS
3
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
7
0
7
Likely Pathogenic
0
0
3
0
3
VUS
0
48
1
0
49
Likely Benign
0
1
1
1
3
Benign
0
0
0
0
0
Total04912162

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

View in ClinVar →

Protein Context — Lollipop Plot

CEBPB · 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.

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Clinical Literature
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