NAT2

Chr 8AR

N-acetyltransferase 2

The encoded enzyme catalyzes N- or O-acetylation of arylamine and hydrazine substrates, participating in drug detoxification and carcinogen bioactivation. Mutations cause slow acetylation phenotype, which affects drug metabolism rates and is associated with increased cancer risk and drug toxicity. This follows autosomal recessive inheritance and shows low constraint against loss-of-function variants.

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

[Acetylation, slow]MIM #243400
AR
3
Active trials
69
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.79
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryNAT2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
3 active or recruiting trials — potential therapeutic options may be available
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.79LOEUF
pLI 0.000
Z-score -0.22
OE 1.09 (0.631.79)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-1.38Z-score
OE missense 1.32 (1.171.49)
196 obs / 148.6 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.09 (0.631.79)
00.351.4
Missense OE1.32 (1.171.49)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 8 / 7.4Missense obs/exp: 196 / 148.6Syn Z: -0.14
DN
0.6938th %ile
GOF
0.6931th %ile
LOF
0.1697th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NAT2 · protein map & ClinVar variants

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

Clinical Literature
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