ZNF516

Chr 18

zinc finger protein 516

Also known as: HsT287

This protein functions as a transcriptional regulator that activates genes involved in brown adipose tissue differentiation and thermogenesis, and may play a role in cellular response to replication stress. Mutations in ZNF516 cause autosomal recessive developmental and epileptic encephalopathy with movement abnormalities, typically presenting in infancy. The gene is highly constrained against loss-of-function variation in the general population.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
5
Pubs (1 yr)
160
P/LP submissions
0%
P/LP missense
LOEUF
LOF
Mechanism· predicted
Clinical SummaryZNF516
📋
ClinVar Variants
158 unique Pathogenic / Likely Pathogenic· 213 VUS of 406 total submissions
Some data sources returned errors (1)

gnomad: TimeoutError: The operation was aborted due to timeout

Population Genetics & Constraint

Constraint data not available from gnomAD.

DN
0.2499th %ile
GOF
0.2298th %ile
LOF
0.85top 5%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

406 submitted variants in ClinVar

Classification Summary

Pathogenic151
Likely Pathogenic7
VUS213
Likely Benign15
Benign4
Conflicting1
151
Pathogenic
7
Likely Pathogenic
213
VUS
15
Likely Benign
4
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
151
0
151
Likely Pathogenic
0
0
7
0
7
VUS
0
209
4
0
213
Likely Benign
0
13
0
2
15
Benign
0
0
1
3
4
Conflicting
1
Total02221635391

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

View in ClinVar →

Protein Context — Lollipop Plot

ZNF516 · 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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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗