ZNF500

Chr 16

zinc finger protein 500

Also known as: ZKSCAN18, ZSCAN50

This nuclear transcription factor regulates gene expression by binding to specific DNA sequences and modulating RNA polymerase II-mediated transcription. The gene shows very low constraint against loss-of-function variants (pLI ~0, LOEUF 0.949), and no clear disease associations have been established in the provided data. Clinical significance of variants in this gene remains uncertain.

ResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 0.95
Clinical SummaryZNF500
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
0.95LOEUF
pLI 0.000
Z-score 1.73
OE 0.57 (0.360.95)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-1.67Z-score
OE missense 1.26 (1.161.37)
397 obs / 313.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.57 (0.360.95)
00.351.4
Missense OE1.26 (1.161.37)
00.61.4
Synonymous OE1.40
01.21.6
LoF obs/exp: 11 / 19.2Missense obs/exp: 397 / 313.9Syn Z: -3.73
DN
0.7132th %ile
GOF
0.73top 25%
LOF
0.2969th %ile

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

GOFprediction above median
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

ZNF500 · 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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