NFASC

Chr 1AR

neurofascin

Also known as: NEDCPMD, NF, NRCAML

This protein is a cell adhesion molecule that organizes the axon initial segment and nodes of Ranvier by clustering voltage-gated sodium channels and linking the extracellular matrix to the intracellular cytoskeleton. Biallelic mutations cause autosomal recessive neurodevelopmental disorder with central and peripheral motor dysfunction. The gene is highly constrained against loss-of-function variants, indicating that heterozygous loss alone is typically tolerated.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.211 OMIM phenotype
Clinical SummaryNFASC
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.
📋
ClinVar Variants
29 unique Pathogenic / Likely Pathogenic· 213 VUS of 371 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.21LOEUF
pLI 1.000
Z-score 6.69
OE 0.12 (0.070.21)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
2.59Z-score
OE missense 0.74 (0.690.79)
588 obs / 793.1 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.12 (0.070.21)
00.351.4
Missense OE0.74 (0.690.79)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 8 / 67.1Missense obs/exp: 588 / 793.1Syn Z: 0.22
DN
0.4983th %ile
GOF
0.5366th %ile
LOF
0.64top 25%

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

LOFprediction above median · 28% of P/LP variants are LoF · LOEUF 0.21

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

371 submitted variants in ClinVar

Classification Summary

Pathogenic23
Likely Pathogenic6
VUS213
Likely Benign69
Benign21
Conflicting8
23
Pathogenic
6
Likely Pathogenic
213
VUS
69
Likely Benign
21
Benign
8
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
3
5
15
0
23
Likely Pathogenic
5
0
1
0
6
VUS
4
199
10
0
213
Likely Benign
0
29
11
29
69
Benign
0
3
2
16
21
Conflicting
8
Total122363945340

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

View in ClinVar →

Protein Context — Lollipop Plot

NFASC · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC