ZNF174

Chr 16

zinc finger protein 174

Also known as: ZSCAN8

This protein functions as a transcriptional repressor containing three zinc fingers and a SCAN domain that enables homodimer formation. The gene shows low constraint against loss-of-function variants (pLI 0.01, LOEUF 0.71), and no definitive human disease associations have been established for ZNF174 mutations. Further research is needed to determine if variants in this gene cause pediatric neurological conditions.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.70
Clinical SummaryZNF174
Population Constraint (gnomAD)
Low constraint (pLI 0.01) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
34 unique Pathogenic / Likely Pathogenic· 77 VUS of 119 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.70LOEUF
pLI 0.015
Z-score 2.44
OE 0.36 (0.200.70)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.56Z-score
OE missense 0.90 (0.801.01)
205 obs / 228.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.36 (0.200.70)
00.351.4
Missense OE0.90 (0.801.01)
00.61.4
Synonymous OE1.29
01.21.6
LoF obs/exp: 6 / 16.8Missense obs/exp: 205 / 228.8Syn Z: -2.09
DN
0.74top 25%
GOF
0.5465th %ile
LOF
0.3453th %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

119 submitted variants in ClinVar

Classification Summary

Pathogenic33
Likely Pathogenic1
VUS77
Likely Benign3
33
Pathogenic
1
Likely Pathogenic
77
VUS
3
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
33
0
33
Likely Pathogenic
0
0
1
0
1
VUS
0
61
16
0
77
Likely Benign
1
0
0
2
3
Benign
0
0
0
0
0
Total161502114

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

ZNF174 · 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 →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC
The SCAN domain of ZNF174 is a dimer.
Stone JR et al.·J Biol Chem
2002