CLUAP1

Chr 16

intraflagellar transport 38

Also known as: CFAP22, CLUAP1, FAP22

The protein is required for cilia biogenesis and functions within the intraflagellar transport complex B (IFT-B) as a key regulator of hedgehog signaling. Mutations cause autosomal recessive ciliopathies including Joubert syndrome and nephronophthisis, which are multisystem disorders affecting the brain, kidneys, and other organs. The gene shows low constraint to loss-of-function variants (pLI = 0.0004), consistent with its recessive inheritance pattern.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.68
Clinical SummaryCLUAP1
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
0.68LOEUF
pLI 0.000
Z-score 2.76
OE 0.40 (0.250.68)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.04Z-score
OE missense 0.99 (0.891.11)
229 obs / 230.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.40 (0.250.68)
00.351.4
Missense OE0.99 (0.891.11)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 10 / 24.8Missense obs/exp: 229 / 230.9Syn Z: -0.38
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongIFT38-related Leber congenital amaurosisOTHERAR
strongIFT38-related ciliopathy syndromeLOFAR

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

DN
0.83top 10%
GOF
0.5954th %ile
LOF
0.2581th %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

CLUAP1 · 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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