CEP43

Chr 6

centrosomal protein 43

Also known as: FGFR1OP, FOP

This gene encodes a centrosomal protein that anchors microtubules to centrosomes and is required for ciliation. Mutations cause myeloproliferative disorder through chromosomal translocations with FGFR1, with inheritance pattern not clearly established from available data. The gene shows low constraint to loss-of-function variants, suggesting tolerance to protein disruption.

OMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismLOEUF 0.731 OMIM phenotype
Clinical SummaryCEP43
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.73LOEUF
pLI 0.001
Z-score 2.50
OE 0.42 (0.250.73)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.19Z-score
OE missense 1.04 (0.931.17)
199 obs / 191.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.42 (0.250.73)
00.351.4
Missense OE1.04 (0.931.17)
00.61.4
Synonymous OE0.97
01.21.6
LoF obs/exp: 9 / 21.5Missense obs/exp: 199 / 191.5Syn Z: 0.22
DN
0.74top 25%
GOF
0.4184th %ile
LOF
0.3162th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction 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

CEP43 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found