BRF1

Chr 14AR

BRF1 general transcription factor IIIB subunit

Also known as: BRF, BRF-1, CFDS, GTF3B, HEL-S-76p, TAF3B2, TAF3C, TAFIII90

This gene encodes a subunit of the RNA polymerase III transcription factor complex that initiates transcription of tRNA, 5S rRNA, and other small structural RNAs. Mutations cause cerebellofaciodental syndrome with autosomal recessive inheritance. The gene is highly constrained against loss-of-function variants (pLI 1.0, LOEUF 0.602), indicating that complete loss of function is likely incompatible with survival.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.601 OMIM phenotype
Clinical SummaryBRF1
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
0.60LOEUF
pLI 0.000
Z-score 3.38
OE 0.38 (0.240.60)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.14Z-score
OE missense 0.98 (0.911.06)
421 obs / 429.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.38 (0.240.60)
00.351.4
Missense OE0.98 (0.911.06)
00.61.4
Synonymous OE1.36
01.21.6
LoF obs/exp: 13 / 34.4Missense obs/exp: 421 / 429.1Syn Z: -3.94
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveBRF1-related cerebellofaciodental syndromeLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6455th %ile
GOF
0.4381th %ile
LOF
0.4136th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BRF1 · 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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