FGFBP2

Chr 4

fibroblast growth factor binding protein 2

Also known as: HBP17RP, KSP37

The protein is a fibroblast growth factor binding protein that is secreted by cytotoxic lymphocytes and functions in cytotoxic lymphocyte-mediated immunity. FGFBP2 mutations cause autosomal recessive developmental and epileptic encephalopathy with onset in infancy, characterized by severe intellectual disability, seizures, and brain malformations. The gene is not highly constrained against loss-of-function variants, consistent with a recessive inheritance pattern.

Summary from RefSeq
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0
Active trials
11
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.92
LOEUF
DN
Mechanism· predicted
Clinical SummaryFGFBP2
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
1.92LOEUF
pLI 0.004
Z-score -0.48
OE 1.35 (0.531.92)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.04Z-score
OE missense 1.01 (0.881.17)
132 obs / 130.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.35 (0.531.92)
00.351.4
Missense OE1.01 (0.881.17)
00.61.4
Synonymous OE1.12
01.21.6
LoF obs/exp: 3 / 2.2Missense obs/exp: 132 / 130.7Syn Z: -0.69
DN
0.6261th %ile
GOF
0.3986th %ile
LOF
0.3163th %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

FGFBP2 · 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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