CEP120

Chr 5AR

centrosomal protein 120

Also known as: CCDC100, JBTS31, SRTD13

This gene encodes a protein that functions in the microtubule-dependent coupling of the nucleus and the centrosome. A similar protein in mouse plays a role in both interkinetic nuclear migration, which is a characteristic pattern of nuclear movement in neural progenitors, and in neural progenitor self-renewal. Mutations in this gene are predicted to result in neurogenic defects. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Oct 2009]

OMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 0.802 OMIM phenotypes
Clinical SummaryCEP120
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Gene-Disease Validity (ClinGen)
ciliopathy · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.80LOEUF
pLI 0.000
Z-score 2.78
OE 0.59 (0.450.80)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.23Z-score
OE missense 1.03 (0.961.11)
525 obs / 510.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.59 (0.450.80)
00.351.4
Missense OE?1.03 (0.961.11)
00.61.4
Synonymous OE?1.02
01.21.6
LoF obs/exp: 32 / 54.1Missense obs/exp: 525 / 510.2Syn Z: -0.23
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveCEP120-related ciliopathy syndromeLOFAR

This gene — mechanism propensity

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6743th %ile
GOF
0.5758th %ile
LOF
0.3647th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CEP120 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.