ZNF706

Chr 8

zinc finger protein 706

Also known as: HSPC038, PNAS-106, PNAS-113

ZNF706 encodes a transcription repressor that binds zinc ions and negatively regulates DNA transcription, specifically repressing KLF4 expression to control embryonic stem cell exit from self-renewal. Mutations cause autosomal recessive developmental and epileptic encephalopathy with severe intellectual disability and early-onset seizures. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.75), suggesting some tolerance to complete gene loss.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
3
Pubs (1 yr)
38
P/LP submissions
P/LP missense
0.75
LOEUF
Mechanism
Clinical SummaryZNF706
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.70) — some intolerance to loss-of-function variants.
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ClinVar Variants
38 unique Pathogenic / Likely Pathogenic· 5 VUS of 52 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.75LOEUF
pLI 0.702
Z-score 1.85
OE 0.00 (0.000.75)
Moderately constrained

Typical tolerance to LoF variation

Missense Constraint
1.76Z-score
OE missense 0.21 (0.120.37)
8 obs / 38.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.000.75)
00.351.4
Missense OE0.21 (0.120.37)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 0 / 4.0Missense obs/exp: 8 / 38.9Syn Z: -0.22

ClinVar Variant Classifications

52 submitted variants in ClinVar

Classification Summary

Pathogenic37
Likely Pathogenic1
VUS5
37
Pathogenic
1
Likely Pathogenic
5
VUS

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
37
Likely Pathogenic
1
VUS
5
Likely Benign
0
Benign
0
Total43

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

ZNF706 · 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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Clinical Literature
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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC