BDP1

Chr 5AR

BDP1 general transcription factor IIIB subunit

Also known as: DFNB112, HSA238520, TAF3B1, TFC5, TFIIIB'', TFIIIB150, TFIIIB90, TFNR

BDP1 encodes a subunit of the TFIIIB transcription initiation complex that is required for RNA polymerase III transcription from all three types of promoters. Mutations cause autosomal recessive deafness-112, though the phenotypic spectrum may be incompletely characterized given the gene's essential role in transcription. The gene is highly constrained against loss-of-function variants (LOEUF 0.596), suggesting mutations may have broader developmental consequences.

OMIMResearchSummary from RefSeq, OMIM, UniProt
ARLOEUF 0.601 OMIM phenotype
Clinical SummaryBDP1
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Gene-Disease Validity (ClinGen)
nonsyndromic genetic hearing loss · ARLimited

Limited evidence — not for standalone diagnostic reporting

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

Moderate LoF intolerance
LoF Constraint
0.60LOEUF
pLI 0.000
Z-score 5.22
OE 0.48 (0.380.60)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
-0.12Z-score
OE missense 1.01 (0.961.06)
1314 obs / 1301.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.48 (0.380.60)
00.351.4
Missense OE1.01 (0.961.06)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 55 / 115.5Missense obs/exp: 1314 / 1301.4Syn Z: -1.25

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BDP1 · 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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