TBL1Y

Chr YYL

transducin beta like 1 Y-linked

Also known as: DFNY2, TBL1

The protein is an F-box-like protein that recruits the ubiquitin/19S proteasome complex to nuclear receptor-regulated transcription units and plays an essential role in transcription activation by mediating proteasomal degradation of transcription repressor complexes. Mutations cause Y-linked deafness type 2, which follows Y-linked inheritance (passed from father to son). The gene shows low constraint against loss-of-function variants.

OMIMResearchSummary from RefSeq, OMIM, UniProt
YLLOEUF 1.961 OMIM phenotype
Clinical SummaryTBL1Y
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
1.96LOEUF
pLI 0.000
Z-score -2.18
OE 1.82 (1.131.96)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-3.77Z-score
OE missense 2.15 (1.791.99)
183 obs / 85.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.82 (1.131.96)
00.351.4
Missense OE2.15 (1.791.99)
00.61.4
Synonymous OE2.01
01.21.6
LoF obs/exp: 15 / 8.2Missense obs/exp: 183 / 85.2Syn Z: -4.76

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TBL1Y · 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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Key Publications
Landmark & review papers · by relevance
PubMed
Top 3 results · since 2015Search PubMed ↗