TAF3

Chr 10

TATA-box binding protein associated factor 3

Also known as: TAF140, TAFII-140, TAFII140

TAF3 encodes a subunit of the TFIID transcription factor complex that recognizes promoters and initiates RNA polymerase II-dependent transcription, and also functions in muscle cell differentiation by forming complexes that replace TFIID at specific promoters. Mutations cause autosomal recessive intellectual disability with dysmorphic facies and behavioral abnormalities. This gene is highly constrained against loss-of-function variants, indicating that such mutations are likely pathogenic.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
5
Pubs (1 yr)
30
P/LP submissions
0%
P/LP missense
0.14
LOEUF· LoF intol.
LOF
Mechanism· predicted
Clinical SummaryTAF3
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
30 unique Pathogenic / Likely Pathogenic· 98 VUS of 147 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.14LOEUF
pLI 1.000
Z-score 5.27
OE 0.03 (0.010.14)
Highly constrained

Among the most LoF-intolerant genes (~top 3%)

Missense Constraint
2.21Z-score
OE missense 0.72 (0.660.79)
360 obs / 498.6 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.03 (0.010.14)
00.351.4
Missense OE0.72 (0.660.79)
00.61.4
Synonymous OE1.01
01.21.6
LoF obs/exp: 1 / 34.3Missense obs/exp: 360 / 498.6Syn Z: -0.15
DN
0.17100th %ile
GOF
0.12100th %ile
LOF
0.91top 5%

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

LOFprediction above median · LOEUF 0.14

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

147 submitted variants in ClinVar

Classification Summary

Pathogenic28
Likely Pathogenic2
VUS98
Likely Benign4
Benign4
28
Pathogenic
2
Likely Pathogenic
98
VUS
4
Likely Benign
4
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
28
0
28
Likely Pathogenic
0
0
2
0
2
VUS
0
88
10
0
98
Likely Benign
0
4
0
0
4
Benign
0
1
1
2
4
Total093412136

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

View in ClinVar →

Protein Context — Lollipop Plot

TAF3 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

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