CEP83

Chr 12AR

centrosomal protein 83

Also known as: CCDC41, NPHP18, NY-REN-58

The encoded protein is a centriolar component that initiates primary cilium assembly and collaborates with IFT20 in trafficking ciliary membrane proteins from the Golgi complex to the cilium. Mutations cause nephronophthisis 18, an infantile kidney disorder that can be associated with intellectual disability. The condition follows autosomal recessive inheritance.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.891 OMIM phenotype
Clinical SummaryCEP83
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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GeneReview available — CEP83
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.89LOEUF
pLI 0.000
Z-score 2.12
OE 0.65 (0.480.89)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.41Z-score
OE missense 0.94 (0.851.03)
316 obs / 337.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.65 (0.480.89)
00.351.4
Missense OE0.94 (0.851.03)
00.61.4
Synonymous OE1.11
01.21.6
LoF obs/exp: 28 / 43.0Missense obs/exp: 316 / 337.0Syn Z: -0.95
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveCEP83-related infantile nephronophthisis and intellectual disabilityLOFAR

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

DN
0.76top 25%
GOF
0.6442th %ile
LOF
0.3261th %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

CEP83 · 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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