CETN2

Chr X

centrin 2

Also known as: CALT, CEN2

The CETN2 protein is a calcium-binding structural component of the centrosome that is required for centriole duplication, correct spindle formation, and cytokinesis. Dominant-negative mutations in CETN2 cause disrupted centrosome function, leading to defects in cell division and genome stability. The associated clinical phenotypes and inheritance pattern have not been established in the provided data.

Summary from RefSeq, UniProt, Mechanism
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0
Active trials
3
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.78
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryCETN2
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.50) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.78LOEUF
pLI 0.503
Z-score 1.91
OE 0.16 (0.060.78)
Moderately constrained

Typical tolerance to LoF variation

Missense Constraint
1.50Z-score
OE missense 0.47 (0.350.64)
30 obs / 63.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.16 (0.060.78)
00.351.4
Missense OE0.47 (0.350.64)
00.61.4
Synonymous OE0.73
01.21.6
LoF obs/exp: 1 / 6.1Missense obs/exp: 30 / 63.5Syn Z: 0.99
DN
0.77top 25%
GOF
0.6443th %ile
LOF
0.3746th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CETN2 · 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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