NT5C2

Chr 10AR

5'-nucleotidase, cytosolic II

Also known as: GMP, NT5B, PNT5, SPG45, SPG65, cN-II

The protein encoded by this gene is a cytosolic 5'-nucleotidase that dephosphorylates purine nucleoside monophosphates, particularly IMP and GMP, and also functions as a phosphotransferase to regulate cellular purine nucleotide pools. Mutations cause spastic paraplegia 45, an autosomal recessive disorder. The pathogenic mechanism likely involves disruption of purine metabolism leading to spasticity and progressive weakness of the lower limbs.

OMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismARLOEUF 0.701 OMIM phenotype
Clinical SummaryNT5C2
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Gene-Disease Validity (ClinGen)
complex hereditary spastic paraplegia · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

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
0.70LOEUF
pLI 0.000
Z-score 2.88
OE 0.46 (0.300.70)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
2.64Z-score
OE missense 0.58 (0.510.66)
181 obs / 312.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.46 (0.300.70)
00.351.4
Missense OE0.58 (0.510.66)
00.61.4
Synonymous OE1.09
01.21.6
LoF obs/exp: 15 / 32.8Missense obs/exp: 181 / 312.5Syn Z: -0.76
DN
0.6454th %ile
GOF
0.6149th %ile
LOF
0.2678th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction 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

NT5C2 · 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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