TUT1

Chr 11

terminal uridylyl transferase 1, U6 snRNA-specific

Also known as: PAPD2, RBM21, STARPAP, TENT1, TUTase, URLC6

This protein functions as a poly(A) polymerase that adds nucleotides to the 3' end of specific mRNAs and also acts as a terminal uridylyltransferase for U6 snRNA, playing essential roles in mRNA processing and gene expression control. Mutations cause autosomal recessive intellectual disability with onset in early childhood. The gene shows strong constraint against loss-of-function variants, indicating that complete loss of protein function is likely incompatible with normal development.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.83
Clinical SummaryTUT1
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
0.83LOEUF
pLI 0.000
Z-score 2.41
OE 0.58 (0.420.83)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.42Z-score
OE missense 0.95 (0.881.02)
504 obs / 531.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.58 (0.420.83)
00.351.4
Missense OE0.95 (0.881.02)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 23 / 39.3Missense obs/exp: 504 / 531.4Syn Z: -0.61

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TUT1 · 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
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 4 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC