TUT7

Chr 9

terminal uridylyl transferase 7

This uridylyltransferase regulates mRNA stability and microRNA biogenesis through terminal uridylation of RNA substrates, playing critical roles in post-transcriptional gene regulation. Mutations cause autosomal recessive intellectual disability with onset in early childhood. The gene is highly constrained against loss-of-function variants (pLI >0.99, LOEUF 0.11), indicating intolerance to functional disruption.

OMIMResearchSummary from UniProt
LOFmechanismLOEUF 0.11
Clinical SummaryTUT7
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.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.11LOEUF
pLI 1.000
Z-score 7.56
OE 0.04 (0.020.11)
Highly constrained

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

Missense Constraint
2.73Z-score
OE missense 0.72 (0.670.78)
558 obs / 771.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.04 (0.020.11)
00.351.4
Missense OE0.72 (0.670.78)
00.61.4
Synonymous OE0.90
01.21.6
LoF obs/exp: 3 / 72.4Missense obs/exp: 558 / 771.5Syn Z: 1.32
DN
0.3693th %ile
GOF
0.3293th %ile
LOF
0.66top 25%

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

LOFprediction above median · LOEUF 0.11

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

TUT7 · 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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