FCN2

Chr 9

ficolin 2

Also known as: EBP-37, FCNL, P35, ficolin-2

FCN2 encodes ficolin-2, a calcium-dependent lectin that functions as a pattern recognition receptor, binding carbohydrates on pathogen surfaces and initiating the lectin pathway of complement activation to enhance immune responses. Mutations cause ficolin-2 deficiency, an autosomal recessive primary immunodeficiency that predisposes to recurrent infections, particularly respiratory tract infections. The gene is highly tolerant to loss-of-function variants (very low pLI), consistent with the recessive inheritance pattern observed in clinical practice.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 1.53
Clinical SummaryFCN2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
45 unique Pathogenic / Likely Pathogenic· 65 VUS of 119 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.53LOEUF
pLI 0.000
Z-score -0.03
OE 1.01 (0.681.53)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.36Z-score
OE missense 1.07 (0.961.21)
204 obs / 190.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.01 (0.681.53)
00.351.4
Missense OE1.07 (0.961.21)
00.61.4
Synonymous OE1.11
01.21.6
LoF obs/exp: 16 / 15.9Missense obs/exp: 204 / 190.0Syn Z: -0.77
DN
0.7229th %ile
GOF
0.6541th %ile
LOF
0.2970th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction 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

119 submitted variants in ClinVar

Classification Summary

Pathogenic43
Likely Pathogenic2
VUS65
Likely Benign2
Benign7
43
Pathogenic
2
Likely Pathogenic
65
VUS
2
Likely Benign
7
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
43
0
43
Likely Pathogenic
1
0
1
0
2
VUS
0
52
13
0
65
Likely Benign
0
1
0
1
2
Benign
0
5
1
1
7
Total158582119

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

View in ClinVar →

Protein Context — Lollipop Plot

FCN2 · 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
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