ZSCAN10

Chr 16AR

zinc finger and SCAN domain containing 10

The protein is an embryonic stem cell-specific transcription factor that maintains pluripotency by binding specific DNA sequences and regulating expression of key genes including POU5F1/OCT4. Mutations cause otofacial neurodevelopmental syndrome with autosomal recessive inheritance. The gene is highly constrained against loss-of-function variants (pLI near 1, LOEUF 0.733), indicating intolerance to complete protein loss.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.731 OMIM phenotype
Clinical SummaryZSCAN10
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

ncbi: Error: NCBI fetch failed: 429 https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esearch.fcgi

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.73LOEUF
pLI 0.000
Z-score 2.57
OE 0.44 (0.280.73)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.51Z-score
OE missense 0.94 (0.871.01)
456 obs / 487.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.44 (0.280.73)
00.351.4
Missense OE0.94 (0.871.01)
00.61.4
Synonymous OE1.10
01.21.6
LoF obs/exp: 11 / 24.9Missense obs/exp: 456 / 487.5Syn Z: -1.14
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
moderateZSCAN10-related neurodevelopmental disorder with oto-facial malformationsLOFAR

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

DN
0.88top 5%
GOF
0.82top 10%
LOF
0.1697th %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

ZSCAN10 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →