THUMPD1

Chr 16AR

THUMP domain 1 NAT10 acetyltransferase adaptor

Also known as: NEDSOA, Tan1

The protein functions as a tRNA-binding adapter that mediates tRNA acetylation by facilitating the modification of cytidine to N4-acetylcytidine. Mutations cause autosomal recessive neurodevelopmental disorder with speech delay and variable ocular anomalies. This gene is highly intolerant to loss-of-function variants, indicating its critical role in normal development.

OMIMResearchSummary from RefSeq, OMIM, UniProt
ARLOEUF 1.581 OMIM phenotype
Clinical SummaryTHUMPD1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
22 unique Pathogenic / Likely Pathogenic· 63 VUS of 94 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.58LOEUF
pLI 0.000
Z-score -0.21
OE 1.06 (0.721.58)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.48Z-score
OE missense 0.90 (0.801.02)
169 obs / 187.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.06 (0.721.58)
00.351.4
Missense OE0.90 (0.801.02)
00.61.4
Synonymous OE0.91
01.21.6
LoF obs/exp: 17 / 16.1Missense obs/exp: 169 / 187.4Syn Z: 0.59

ClinVar Variant Classifications

94 submitted variants in ClinVar

Classification Summary

Pathogenic21
Likely Pathogenic1
VUS63
Likely Benign3
Conflicting1
21
Pathogenic
1
Likely Pathogenic
63
VUS
3
Likely Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
6
1
14
0
21
Likely Pathogenic
0
1
0
0
1
VUS
1
56
6
0
63
Likely Benign
0
2
1
0
3
Benign
0
0
0
0
0
Conflicting
1
Total76021089

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

THUMPD1 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →