SPATA6

Chr 1

spermatogenesis associated 6

Also known as: HASH, SRF-1, SRF1

The protein is required for formation of the sperm connecting piece during spermiogenesis and binds myosin light chains to facilitate motile cilium assembly. Mutations cause autosomal recessive primary ciliary dyskinesia with reduced generation of multiple motile cilia, presenting with respiratory symptoms, laterality defects, and male infertility. The gene shows very low constraint against loss-of-function variants (pLI near 0), consistent with autosomal recessive inheritance where heterozygous carriers are unaffected.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.47
Clinical SummarySPATA6
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
1.47LOEUF
pLI 0.000
Z-score -0.41
OE 1.08 (0.811.47)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.20Z-score
OE missense 0.97 (0.871.07)
251 obs / 260.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.08 (0.811.47)
00.351.4
Missense OE0.97 (0.871.07)
00.61.4
Synonymous OE0.85
01.21.6
LoF obs/exp: 30 / 27.7Missense obs/exp: 251 / 260.0Syn Z: 1.15
DN
0.6842th %ile
GOF
0.5857th %ile
LOF
0.4825th %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

SPATA6 · 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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