NTMT1

Chr 9

N-terminal Xaa-Pro-Lys N-methyltransferase 1

Also known as: AD-003, C9orf32, HOMT1A, METTL11A, NRMT, NRMT1, NTM1A

The protein functions as a distributive alpha-N-methyltransferase that methylates the N-terminus of target proteins containing specific motifs, including key substrates like RCC1, SET, and RB1, and is required for normal bipolar spindle formation and chromosome segregation during mitosis. Based on the tolerance metrics provided (pLI: 0.05, LOEUF: 1.077), this gene appears relatively tolerant to loss-of-function variation, but specific disease associations and inheritance patterns are not established in the provided data.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.08
Clinical SummaryNTMT1
Population Constraint (gnomAD)
Low constraint (pLI 0.05) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
34 unique Pathogenic / Likely Pathogenic· 37 VUS of 96 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.08LOEUF
pLI 0.051
Z-score 1.45
OE 0.42 (0.191.08)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
1.29Z-score
OE missense 0.70 (0.600.83)
104 obs / 148.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.42 (0.191.08)
00.351.4
Missense OE0.70 (0.600.83)
00.61.4
Synonymous OE1.10
01.21.6
LoF obs/exp: 3 / 7.2Missense obs/exp: 104 / 148.1Syn Z: -0.67

ClinVar Variant Classifications

96 submitted variants in ClinVar

Classification Summary

Pathogenic32
Likely Pathogenic2
VUS37
Likely Benign4
32
Pathogenic
2
Likely Pathogenic
37
VUS
4
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
32
0
32
Likely Pathogenic
0
0
2
0
2
VUS
0
29
8
0
37
Likely Benign
0
3
0
1
4
Benign
0
0
0
0
0
Total03242175

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

View in ClinVar →

Protein Context — Lollipop Plot

NTMT1 · 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.

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Clinical Literature
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