NT5M

Chr 17

5',3'-nucleotidase, mitochondrial

Also known as: dNT-2, dNT2, mdN

The protein is a mitochondrial matrix 5' nucleotidase that dephosphorylates uracil and thymine deoxyribonucleotides to protect mitochondrial DNA replication from excess dTTP. Mutations cause autosomal recessive spastic paraplegia-45, which presents with progressive spasticity and weakness of the lower extremities. The gene shows low constraint against loss-of-function variants, consistent with recessive inheritance where heterozygous carriers are typically unaffected.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.85
Clinical SummaryNT5M
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
1.85LOEUF
pLI 0.000
Z-score -0.69
OE 1.25 (0.781.85)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.18Z-score
OE missense 0.95 (0.821.11)
115 obs / 120.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.25 (0.781.85)
00.351.4
Missense OE0.95 (0.821.11)
00.61.4
Synonymous OE1.12
01.21.6
LoF obs/exp: 11 / 8.8Missense obs/exp: 115 / 120.5Syn Z: -0.67

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NT5M · 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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