ZBED1

Chr Y

zinc finger BED-type containing 1

Also known as: ALTE, DREF, TRAMP, hDREF

ZBED1 encodes a transcription factor and SUMO E3 ligase that promotes cell proliferation by binding to promoter regions and activating transcription of genes including histones and ribosomal proteins. The gene is located in the pseudoautosomal region of the X and Y chromosomes and is not highly constrained against loss-of-function variants. Pathogenic variants in ZBED1 have not been definitively associated with human disease.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.78
Clinical SummaryZBED1
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
0.78LOEUF
pLI 0.000
Z-score 2.29
OE 0.45 (0.270.78)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.85Z-score
OE missense 0.89 (0.820.96)
404 obs / 454.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.45 (0.270.78)
00.351.4
Missense OE0.89 (0.820.96)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 9 / 20.1Missense obs/exp: 404 / 454.9Syn Z: -1.00
DN
0.6744th %ile
GOF
0.5562th %ile
LOF
0.2774th %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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ZBED1 · 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
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 2 results · since 2015Search PubMed ↗