KIAA0586

Chr 14AR

KIAA0586

Also known as: JBTS23, SRTD14, Talpid3

This gene encodes a conserved centrosomal protein that functions in ciliogenesis and responds to hedgehog signaling. Mutations in this gene causes Joubert syndrome 23. Alternative splicing results in multiple transcript variants and protein isoforms. [provided by RefSeq, Aug 2016]

OMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 0.722 OMIM phenotypes
Clinical SummaryKIAA0586
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?
0.72LOEUF
pLI 0.000
Z-score 3.44
OE 0.54 (0.420.72)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.37Z-score
OE missense 0.96 (0.911.02)
735 obs / 763.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.54 (0.420.72)
00.351.4
Missense OE?0.96 (0.911.02)
00.61.4
Synonymous OE?1.00
01.21.6
LoF obs/exp: 36 / 66.2Missense obs/exp: 735 / 763.9Syn Z: -0.03
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveKIAA0586-related Joubert syndromeLOFAR

This gene — mechanism propensity

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

DN
0.6647th %ile
GOF
0.6247th %ile
LOF
0.3941th %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

KIAA0586 · 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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