NAA20

Chr 20AR

N-alpha-acetyltransferase 20, NatB catalytic subunit

Also known as: MRT73, NAT3, NAT3P, NAT5, NAT5P, dJ1002M8.1

The NAA20 protein is the catalytic subunit of the NatB complex, which acetylates N-terminal methionine residues of specific peptides and is required for maintaining actomyosin fiber structure and cellular migration. Mutations cause autosomal recessive intellectual developmental disorder. The gene shows relatively low constraint to loss-of-function variants (pLI 0.006, LOEUF 1.03), consistent with its recessive inheritance pattern.

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

Intellectual developmental disorder, autosomal recessive 73MIM #619717
AR
0
Active trials
5
Pubs (1 yr)
27
P/LP submissions
8%
P/LP missense
1.03
LOEUF
GOF
Mechanism· predicted
Clinical SummaryNAA20
Population Constraint (gnomAD)
Low constraint (pLI 0.01) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
26 unique Pathogenic / Likely Pathogenic· 20 VUS of 53 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.03LOEUF
pLI 0.006
Z-score 1.52
OE 0.49 (0.251.03)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
1.31Z-score
OE missense 0.63 (0.520.78)
65 obs / 102.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.49 (0.251.03)
00.351.4
Missense OE0.63 (0.520.78)
00.61.4
Synonymous OE1.11
01.21.6
LoF obs/exp: 5 / 10.2Missense obs/exp: 65 / 102.4Syn Z: -0.55
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedNAA20-related developmental delay and microcephalyOTHERAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.5967th %ile
GOF
0.72top 25%
LOF
0.2873th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

53 submitted variants in ClinVar

Classification Summary

Pathogenic22
Likely Pathogenic4
VUS20
Likely Benign1
22
Pathogenic
4
Likely Pathogenic
20
VUS
1
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
1
21
0
22
Likely Pathogenic
0
1
3
0
4
VUS
0
16
4
0
20
Likely Benign
0
0
0
1
1
Benign
0
0
0
0
0
Total01828147

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

View in ClinVar →

Protein Context — Lollipop Plot

NAA20 · 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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