ZGPAT

Chr 20

zinc finger CCCH-type and G-patch domain containing

Also known as: GPATC6, GPATCH6, KIAA1847, ZC3H9, ZC3HDC9, ZIP

ZGPAT encodes a transcription repressor that binds specific DNA sequences and recruits the NuRD chromatin complex to repress target gene expression, including negative regulation of EGFR signaling. Biallelic mutations cause autosomal recessive White-Sutton syndrome, characterized by intellectual disability, developmental delay, and distinctive facial features with typical onset in early childhood. The gene shows tolerance to loss-of-function variants (pLI 0.0004), consistent with the recessive inheritance pattern observed clinically.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
3
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.78
LOEUF
DN
Mechanism· predicted
Clinical SummaryZGPAT
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 2.31
OE 0.44 (0.270.78)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.21Z-score
OE missense 0.97 (0.881.06)
310 obs / 320.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.44 (0.270.78)
00.351.4
Missense OE0.97 (0.881.06)
00.61.4
Synonymous OE1.03
01.21.6
LoF obs/exp: 9 / 20.2Missense obs/exp: 310 / 320.5Syn Z: -0.27
DN
0.6161th %ile
GOF
0.5758th %ile
LOF
0.2968th %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

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