NTAQ1

Chr 8

N-terminal glutamine amidase 1

Also known as: C8orf32, WDYHV1

The NTAQ1 protein mediates deamidation of N-terminal glutamine residues to glutamate, facilitating protein degradation through the N-end rule pathway. Mutations cause autosomal recessive developmental and epileptic encephalopathy with movement abnormalities, typically presenting in early infancy with seizures, developmental delay, and dystonic movements. The gene shows minimal constraint against loss-of-function variants (pLI near 0), consistent with recessive inheritance where single functional copies are generally sufficient.

Summary from RefSeq, UniProt
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0
Active trials
0
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.35
LOEUF
Mechanism
Clinical SummaryNTAQ1
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
1.35LOEUF
pLI 0.000
Z-score 0.71
OE 0.77 (0.471.35)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.34Z-score
OE missense 0.91 (0.771.07)
99 obs / 109.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.77 (0.471.35)
00.351.4
Missense OE0.91 (0.771.07)
00.61.4
Synonymous OE1.04
01.21.6
LoF obs/exp: 9 / 11.6Missense obs/exp: 99 / 109.1Syn Z: -0.22

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NTAQ1 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found