CAMTA2

Chr 17

calmodulin binding transcription activator 2

CAMTA2 encodes a calmodulin-binding transcription activator that regulates gene expression, particularly genes involved in cardiac growth, and may function as a tumor suppressor. Mutations cause autosomal dominant developmental delay, intellectual disability, and congenital heart defects with highly variable expressivity. This gene is highly constrained against loss-of-function variants, indicating that such mutations are likely to be pathogenic when they occur.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.26
Clinical SummaryCAMTA2
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

LoF intolerant — likely haploinsufficient
LoF Constraint
0.26LOEUF
pLI 0.999
Z-score 6.30
OE 0.15 (0.090.26)
Highly constrained

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

Missense Constraint
1.55Z-score
OE missense 0.84 (0.790.90)
627 obs / 746.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.15 (0.090.26)
00.351.4
Missense OE0.84 (0.790.90)
00.61.4
Synonymous OE0.97
01.21.6
LoF obs/exp: 10 / 64.6Missense obs/exp: 627 / 746.5Syn Z: 0.38
DN
0.3693th %ile
GOF
0.4381th %ile
LOF
0.73top 10%

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

LOFprediction above median · LOEUF 0.26

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

CAMTA2 · 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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