TAF1

Chr XXLR

TATA-box binding protein associated factor 1

Also known as: BA2R, CCG1, CCGS, DYT3, DYT3/TAF1, KAT4, MRXS33, N-TAF1

TAF1 encodes the largest subunit of TFIID, a basal transcription factor that binds core promoter sequences and coordinates RNA polymerase II transcription initiation, and possesses kinase, acetyltransferase, and ubiquitin-conjugating activities. Mutations cause X-linked recessive dystonia-parkinsonism (DYT3) and X-linked syndromic intellectual developmental disorder through loss-of-function mechanisms. The gene is highly intolerant to loss-of-function variants, reflecting its essential role in transcriptional regulation.

OMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismXLRLOEUF 0.042 OMIM phenotypes
Clinical SummaryTAF1
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.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.04LOEUF
pLI 1.000
Z-score 7.69
OE 0.00 (0.000.04)
Highly constrained

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

Missense Constraint
5.49Z-score
OE missense 0.44 (0.400.48)
326 obs / 748.3 exp
Constrained

Extremely missense-constrained (top ~0.01%)

Observed / Expected Ratios
LoF OE0.00 (0.000.04)
00.351.4
Missense OE0.44 (0.400.48)
00.61.4
Synonymous OE1.09
01.21.6
LoF obs/exp: 0 / 68.9Missense obs/exp: 326 / 748.3Syn Z: -1.07
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveTAF1-related dysmorphic features, intellectual disability, and neurological manifestationsLOFXLR
DN
0.2399th %ile
GOF
0.3986th %ile
LOF
0.82top 5%

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

LOFprediction above median · LOEUF 0.04

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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TAF1 · 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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