This gene encodes neuroligin-2, a transmembrane scaffolding protein that clusters postsynaptic GABA receptors and modulates inhibitory synaptic signaling. Mutations cause autism spectrum disorder and intellectual disability with autosomal recessive inheritance. The gene is highly constrained against loss-of-function variants, indicating that complete loss of neuroligin-2 function has severe developmental consequences.

GeneReviewsOMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.13
Clinical SummaryNLGN2
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
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ClinVar Variants
28 unique Pathogenic / Likely Pathogenic· 167 VUS of 300 total submissions
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Clinical Trials
2 active or recruiting trials — potential therapeutic options may be available
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GeneReview available — NLGN2
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.13LOEUF
pLI 1.000
Z-score 4.50
OE 0.00 (0.000.13)
Highly constrained

Among the most LoF-intolerant genes (~top 3%)

Missense Constraint
4.47Z-score
OE missense 0.44 (0.400.49)
225 obs / 508.5 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.00 (0.000.13)
00.351.4
Missense OE0.44 (0.400.49)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 0 / 23.6Missense obs/exp: 225 / 508.5Syn Z: 0.28
DN
0.3395th %ile
GOF
0.5758th %ile
LOF
0.69top 10%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.13

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

300 submitted variants in ClinVar

Classification Summary

Pathogenic24
Likely Pathogenic4
VUS167
Likely Benign75
Benign18
Conflicting1
24
Pathogenic
4
Likely Pathogenic
167
VUS
75
Likely Benign
18
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
24
0
24
Likely Pathogenic
0
1
3
0
4
VUS
10
146
10
1
167
Likely Benign
0
3
9
63
75
Benign
0
1
4
13
18
Conflicting
1
Total101515077289

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

View in ClinVar →

Protein Context — Lollipop Plot

NLGN2 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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