FDCSP

Chr 4

follicular dendritic cell secreted protein

Also known as: C4orf7, FDC-SP

This gene encodes a small secreted protein that specifically binds to activated B cells and regulates antibody responses. Mutations in FDCSP are associated with immunodeficiency and developmental disorders, though the specific phenotypic spectrum is still being characterized. The gene shows low constraint against loss-of-function variants, and inheritance patterns for associated disorders remain to be fully established.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.40
Clinical SummaryFDCSP
Population Constraint (gnomAD)
Low constraint (pLI 0.08) — 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
1.40LOEUF
pLI 0.082
Z-score 1.05
OE 0.46 (0.181.40)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.09Z-score
OE missense 1.04 (0.821.32)
48 obs / 46.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.46 (0.181.40)
00.351.4
Missense OE1.04 (0.821.32)
00.61.4
Synonymous OE0.96
01.21.6
LoF obs/exp: 2 / 4.4Missense obs/exp: 48 / 46.3Syn Z: 0.12
DN
0.97top 5%
GOF
0.5953th %ile
LOF
0.04100th %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

FDCSP · 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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