SAXO1

Chr 9

stabilizer of axonemal microtubules 1

The SAXO1 protein binds to and stabilizes microtubules, particularly at low temperatures, and regulates cilium length. Mutations cause autosomal recessive primary ciliary dyskinesia with intellectual disability and developmental delay, affecting the respiratory system and neurodevelopment. This gene is not highly constrained against loss-of-function variants.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 1.85
Clinical SummarySAXO1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.85LOEUF
pLI 0.000
Z-score -1.63
OE 1.40 (1.031.85)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-2.70Z-score
OE missense 1.46 (1.341.58)
402 obs / 275.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.40 (1.031.85)
00.351.4
Missense OE1.46 (1.341.58)
00.61.4
Synonymous OE1.23
01.21.6
LoF obs/exp: 27 / 19.3Missense obs/exp: 402 / 275.7Syn Z: -1.80
DN
0.76top 25%
GOF
0.74top 25%
LOF
0.3163th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction 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

SAXO1 · 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