YY2

Chr X

YY2 transcription factor

Also known as: ZNF631

YY2 encodes a transcription factor containing Kruppel-like zinc fingers that regulates gene expression through both activation and repression domains. Mutations cause autosomal dominant neurodevelopmental disorders with intellectual disability and developmental delay. The gene shows moderate tolerance to loss-of-function variants, suggesting variable penetrance or severity may occur in some cases.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.93
Clinical SummaryYY2
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.20) despite low pLI — interpret in context.
📋
ClinVar Variants
64 unique Pathogenic / Likely Pathogenic· 32 VUS of 105 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.93LOEUF
pLI 0.408
Z-score 1.69
OE 0.20 (0.070.93)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.35Z-score
OE missense 0.71 (0.610.82)
121 obs / 170.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.20 (0.070.93)
00.351.4
Missense OE0.71 (0.610.82)
00.61.4
Synonymous OE1.09
01.21.6
LoF obs/exp: 1 / 5.1Missense obs/exp: 121 / 170.5Syn Z: -0.65

ClinVar Variant Classifications

105 submitted variants in ClinVar

Classification Summary

Pathogenic62
Likely Pathogenic2
VUS32
Likely Benign9
62
Pathogenic
2
Likely Pathogenic
32
VUS
9
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
62
0
62
Likely Pathogenic
0
0
2
0
2
VUS
0
26
6
0
32
Likely Benign
0
8
0
1
9
Benign
0
0
0
0
0
Total034701105

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

View in ClinVar →

Protein Context — Lollipop Plot

YY2 · 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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