NAT10

Chr 11

N-acetyltransferase 10

Also known as: ALP, Kre33, NET43

NAT10 encodes an RNA cytidine acetyltransferase that catalyzes N(4)-acetylcytidine modifications on mRNAs, rRNAs and tRNAs, enhancing mRNA stability and translation efficiency, and is required for early ribosomal RNA processing during ribosome biogenesis. Mutations cause autosomal recessive microcephaly, early-onset seizures, and severe developmental delays. The gene is not highly constrained against loss-of-function variants, consistent with its recessive inheritance pattern.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
171
Pubs (1 yr)
18
P/LP submissions
0%
P/LP missense
0.91
LOEUF
Mechanism
Clinical SummaryNAT10
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
18 unique Pathogenic / Likely Pathogenic· 123 VUS of 186 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.91LOEUF
pLI 0.000
Z-score 2.12
OE 0.71 (0.550.91)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.17Z-score
OE missense 0.86 (0.800.93)
505 obs / 584.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.71 (0.550.91)
00.351.4
Missense OE0.86 (0.800.93)
00.61.4
Synonymous OE0.82
01.21.6
LoF obs/exp: 43 / 60.9Missense obs/exp: 505 / 584.7Syn Z: 2.10

ClinVar Variant Classifications

186 submitted variants in ClinVar

Classification Summary

Pathogenic18
VUS123
Likely Benign3
Benign9
18
Pathogenic
123
VUS
3
Likely Benign
9
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
18
0
18
Likely Pathogenic
0
0
0
0
0
VUS
0
118
4
1
123
Likely Benign
0
2
0
1
3
Benign
0
2
2
5
9
Total0122247153

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

View in ClinVar →

Protein Context — Lollipop Plot

NAT10 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

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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