CGGBP1

Chr 3

CGG triplet repeat binding protein 1

Also known as: CGGBP, p20-CGGBP

The protein binds to unmethylated CGG trinucleotide repeats and regulates transcription, particularly at the FMR1 promoter, while also contributing to DNA damage repair and telomere protection. Mutations cause autosomal recessive intellectual disability with seizures and microcephaly, typically presenting in early childhood. This gene is highly constrained against loss-of-function variants, suggesting that complete loss of protein function is poorly tolerated.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.53
Clinical SummaryCGGBP1
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.82) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.53LOEUF
pLI 0.818
Z-score 2.20
OE 0.00 (0.000.53)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.57Z-score
OE missense 0.55 (0.440.69)
52 obs / 95.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.000.53)
00.351.4
Missense OE0.55 (0.440.69)
00.61.4
Synonymous OE1.21
01.21.6
LoF obs/exp: 0 / 5.6Missense obs/exp: 52 / 95.0Syn Z: -0.95

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CGGBP1 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗